MedWorm: Aplastic Anemia

Cyclophosphamide in aplastic anemia?

Blood — Thu, 18 Mar 2010 16:02:02 +0100

(Source: Blood)

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High-dose cyclophosphamide for severe aplastic anemia: long-term follow-up

Blood — Thu, 18 Mar 2010 16:02:02 +0100

Severe aplastic anemia (SAA) is a life-threatening bone marrow failure disorder that can be treated with bone marrow transplantation, immunosuppressive therapy, and high-dose cyclophosphamide. Here, we report long-term follow-up on 67 SAA patients (44 treatment-naive and 23 refractory) treated with high-dose cyclophosphamide. At 10 years, the overall actuarial survival was 88%, the response rate was 71% with the majority being complete, and the actuarial event-free survival was 58% in 44 treatment-naive SAA patients. Patients with refractory SAA fared less well after high-dose cyclophosphamide therapy; at 10 years, overall actuarial survival, response, and actuarial event-free survival rates were 62%, 48%, and 27%, respectively. High-dose cyclophosphamide is highly effective therapy for se...

Granulocyte transfusions in severe aplastic anemia: an eleven-year experience

The Aspergillus Website - articles — Wed, 17 Mar 2010 00:00:00 +0100

Quillen K, Wong E, Scheinberg P, Young NS, Walsh TJ, Wu CO, Leitman SF (Source: The Aspergillus Website - articles)

Iron chelation therapy associated with improvement of hematopoiesis in transfusion-dependent patients.

Transfusion — Fri, 05 Mar 2010 00:00:00 +0100

CONCLUSION: Although there are few reports on erythroid responses in patients undergoing iron chelation therapy, they may give new insights in the pathogenesis of MDS and other myeloproliferative neoplasms. AA may benefit in terms of erythroid response. The findings in these cases underline the clinical importance of treating patients with iron overload. A survival benefit of chelation in patients with myeloproliferative neoplasms is still to be confirmed. PMID: 20230535 [PubMed - as supplied by publisher] (Source: Transfusion)

Results of hematopoietic stem cell transplant in shiraz: 15 years experience in southern iran.

Mon, 01 Mar 2010 00:00:00 +0100

Conclusions: These data reflect the important role of hematopoietic stem cell transplant in improving survival for a variety of hematopoietic system disorders at our center in Southern Iran. In patients with B-thalassemia major hematopoietic stem cell transplant seems to be the treatment of choice, because it leads to a cure in all classes (Lucarelli risk group, I-III). Based on high success rates in patients with class II and III thalassemia with the addition of the antithymocyte globulin to conditioning regimen of stem cell transplant, we also recommend using this new method of conditioning in transplant of thalassemia patients. PMID: 20199373 [PubMed - in process] (Source: Experimental and Clinical Transplantation : official journal of the Middle East Society for Organ Transplantati...

Long-term follow-up of patients with moderate aplastic anemia and pure red cell aplasia treated with daclizumab.

Haematologica — Mon, 01 Mar 2010 00:00:00 +0100

Conclusions Red cell transfusion-independence prior to treatment in mAA patients predicted response. The only significant adverse treatment-related events were transient rashes and arthralgias. Daclizumab is safe and effective, and produces lengthy remissions in patients with PRCA and mAA. PMID: 20207845 [PubMed - in process] (Source: Haematologica)

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Diagnostic and therapeutic thoracic surgery in leukemia and severe aplastic anemia

The Aspergillus Website - articles — Thu, 18 Feb 2010 00:00:00 +0100

Habicht JM, Gratwohl A, Tamm M, Drewe J, Proske M, Stulz P (Source: The Aspergillus Website - articles)

Progressive osteosclerosis and visceral calcification after cord blood transplantation.

International Journal of Hematology — Wed, 17 Feb 2010 00:00:00 +0100

Authors: Asada N, Ishii S, Wakahashi K, Kawano H, Kawamori Y, Nishikawa S, Minagawa K, Okamura A, Shimoyama M, Katayama Y, Hayashi Y, Itoh T, Tanimoto M, Matsui T A 26-year-old woman, who successfully underwent umbilical cord blood transplantation for aplastic anemia 4 years previously, had suffered from hepatosplenic microabscesses caused by unidentifiable grocott stain-positive spores from immediately after the transplantation. At 51 months post-transplant, we attempted bone marrow biopsy from her posterior iliac crest, but failed to penetrate the cortical bone. X-ray of her spine and pelvis showed marked and diffuse osteosclerosis. Retrospective analysis of computed tomography revealed the gradual replacement of sternal, vertebral, and pelvic bone marrow with calcified tissues in ad...

Proliferation and apoptosis of bone marrow CD4+ T cells in patients with aplastic anemia and impacts of the secreted cytokines on hematopoietic stem cells from umbilical cord blood

Journal of Huazhong University of Science and Technology -- Medical Sciences -- — Sun, 14 Feb 2010 06:41:06 +0100

Summary Recent studies indicate that immune-associated aplastic anemia (AA) resembles such autoimmune diseases as insulin-dependent diabetes and chronic autoimmune thyroiditis that belong to organ-specific autoimmune diseases. Many independent investigation groups have successfully isolated the pathopoiesis-associated T cell clone causing hematopoiesis failure with a CD4 phenotype from peripheral blood and bone marrow (BM) in AA patients. In the current study, BM CD4+ T cells were isolated from AA patients and healthy controls with immunomagnetic beads sorting, and proliferation capability, apoptosis features and the impacts of their secreted cytokines on hematopoiesis stem/progenitor cells were compared between them. By 3H-TdR method, CD4+ T cells in AA group presented mor...

Ciclosporin: Chronic generalised periodontitis in a patient with aplastic anaemia: case report

Reactions — Wed, 10 Feb 2010 14:08:42 +0100

(Source: Reactions)

Multiple synchronous tumors in a child with Fanconi anemia

Journal of Pediatric Surgery — Mon, 01 Feb 2010 00:00:00 +0100

We describe a child who presented with 2 synchronous solid tumors (Wilms tumor and neuroblastoma), later found to have FA, who developed severe toxicity and died after a first cycle of chemotherapy. Our experience emphasizes that a predisposing genetic condition should be sought in cases of multiple tumors and that managing FA patients with cancer can be particularly difficult. (Source: Journal of Pediatric Surgery)

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Telomerase gene mutation screening in Chinese patients with aplastic anemia

Leukemia Research — Sat, 30 Jan 2010 14:33:16 +0100

Abstract: To study the incidence of telomerase gene mutations in Chinese patients with acquired bone marrow failure (BMF) and explore its relationship with telomere shortening. Blood samples from 66 patients with aplastic anemia (AA) in northern China were collected and TERC mutation analysis was performed. Two TERC mutations were identified. The incidence of telomerase gene mutations in Chinese people with acquired AA is similar to that of the western people. (Source: Leukemia Research)

Thymolipoma. A report of nine cases, with emphasis on its association with myasthenia gravis

Surgery Today — Wed, 27 Jan 2010 20:05:29 +0100

Conclusions Thymolipomas are benign tumors that show an excellent outcome. Patients with autoimmune disease symptoms occasionally show an improvement of the symptoms after a resection of the tumors. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00595-009-4042-5Authors Ralf J. Rieker, University Hospital Department of Pathology Heidelberg GermanyPeter Schirmacher, University Hospital Department of Pathology Heidelberg GermanyPhilipp A. Schnabel, University Hospital Department of Pathology Heidelberg GermanyKatharina Moser, Medical University of Innsbruck Department of Pathology Innsbruck AustriaHans Hoffmann, University of Heidelberg Department of Thoracic Surgery, Thoraxklinik Amalienstrasse 5 D-69126 Heidelberg GermanyHendrik Dienemann, University ...

Perioperative management for a patient with chronic pancytopenia: a case of aplastic anemia with persistent neutropenia following preoperative administration of G-CSF

Journal of Anesthesia — Fri, 22 Jan 2010 10:09:15 +0100

Abstract The patient, a 62-year-old male suffering from aplastic anemia for 6 years, was admitted in order to undergo laparoscopic cholecystectomy for cholecystitis. Results of laboratory tests at the time of admission revealed pancytopenia: white blood cell count, 1.94 × 103/µl (neutrophil count, 1.23 × 103/µl); red blood cell count, 2.09 × 106/µl; Hb 7.5 g/dl; and platelet count, 3.7 × 104/µl. The patient received supportive therapy prior to surgery, including blood transfusion of red blood cells, platelets and granulocyte colony-stimulating factor (G-CSF). On the day of surgery, the white blood cell count increased to 3.93 × 103/µl (neutrophil count, 2.75 × 103/µl). Surgery ended with no intr...

Poor potential of proliferation and differentiation in bone marrow mesenchymal stem cells derived from children with severe aplastic anemia

Annals of Hematology — Mon, 18 Jan 2010 18:44:16 +0100

Abstract The pathogenesis of severe aplastic anemia (SAA) has not been completely understood, and insufficiency of the hematopoietic microenvironment can be an important factor. Here, we compared the basic properties of mesenchymal stem cells (MSCs), a major component of bone marrow microenvironment, from five SAA children with those of MSCs from five controls. Although MSCs from SAA children and controls were similar in morphology and immunophenotypic profile, SAA MSCs had slower expansion rate and smaller cumulative population doubling (1.83 ± 1.21 vs 3.36 ± 0.87; p = 0.046), indicating lower proliferative capacity. After osteogenic induction, SAA MSCs showed lower alkaline phosphatase activity (optical density, 1.46 ± 0.04 vs 2.27 ± 0.32; p = ...

In utero and in vitro effects of benzene and its metabolites on erythroid differentiation and the role of reactive oxygen species.

Toxicology and Applied Pharmacology — Fri, 15 Jan 2010 00:00:00 +0100

In conclusion, this study provided evidence that ROS generated as a result of benzene metabolism may significantly alter erythroid differentiation, potentially leading to the development of aplastic anemia. PMID: 20083130 [PubMed - as supplied by publisher] (Source: Toxicology and Applied Pharmacology)

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The value and practicality of granulocyte transfusion: a single oncology centre experience

Transfusion Medicine — Wed, 13 Jan 2010 00:00:00 +0100

summary. There is an increased risk of infection in patients with neutropaenia, especially in those with neutrophil counts of less than 0·5 × 109/L, and neutropaenia-associated infection remains a limiting factor in treating malignancy especially of haematopoietic origin. Transfusing donor neutrophils is a logical approach to these problems, but granulocyte transfusion (GTx), a practice first advocated in the 1960s, is underused and although now enjoying resurgence, remains controversial. The aim of this study was to determine the practical aspects of GTx and clinical responses in patients receiving them. This is an observational retrospective review of GTx in patients undergoing therapy for predominantly haematological malignancies. We reviewed blood bank records and identified patients...

HLA-matched sibling transplantation with BM and CD34+-purified PBSCs in adult patients with high-risk severe aplastic anemia to overcome graft rejection without an increase in GVHD

Bone Marrow Transplantation — Mon, 11 Jan 2010 00:00:00 +0100

HLA-matched sibling transplantation with BM and CD34+-purified PBSCs in adult patients with high-risk severe aplastic anemia to overcome graft rejection without an increase in GVHD Bone Marrow Transplantation advance online publication, January 11, 2010. doi:10.1038/bmt.2009.374 Authors: B S Cho, K S Eom, Y J Kim, H J Kim, S Lee, C K Min, S G Cho, W S Min, C W Park, C C Kim & J W Lee (Source: Bone Marrow Transplantation)

Autoimmunity and the risk of myeloproliferative neoplasms.

Haematologica — Wed, 06 Jan 2010 00:00:00 +0100

Authors: Kristinsson SY, Landgren O, Samuelsson J, Björkholm M, Goldin LR The causes of myeloproliferative neoplasm (MPN) are unknown. We conducted a large population-based study including 11,039 MPN patients and 43,550 matched controls with the aim to assess the associations between a personal history of a broad span of autoimmune diseases and subsequent risk of MPN. We found a prior history of any autoimmune disease to be associated with a significantly increased risk of MPNs (odds ratio (OR)=1.2; 95% confidence interval (CI) 1.0-1.3; p= 0.021). Specifically, we found an increased risk of MPNs associated with a prior immune thrombocytopenic purpura (2.9; 1.7-7.2), Crohn's disease (1.8; 1.1-3.0), polymyalgia rheumatica (1.7; 1.2-2.5), giant cell arteritis (5.9; 2.4-14.4), Reiter'...

Outcomes of matched sibling and alternative donor stem cell transplantation for 26 children with severe aplastic anemia.

International Journal of Hematology — Sat, 26 Dec 2009 00:00:00 +0100

In this study, we reported the outcomes of hematopoietic stem cell transplantation (HSCT) as a treatment modality for pediatric patients admitted over the past 20 years in a single institute. From January 1989 to January 2007, 26 patients with a median age of 8 years underwent 14 matched sibling donor (MSD) and 12 alternative donor (AD) transplantations. Two patients received second transplantation successfully after primary graft failure and late graft rejection. Two patients who received transplantation from matched unrelated donors (MUD) died of sepsis, and one of them also had a graft failure at death. The overall 5-year survival rate was 92.1%: 83.3% for AD transplantation and 100% for MSD transplantation. Grade II acute graft versus host disease (GVHD) developed in 3 patients, and ex...

Clinical study of shengxue mixture (生血合剂) in treating aplastic anemia

Chinese Journal of Integrative Medicine — Wed, 23 Dec 2009 23:08:50 +0100

Conclusions: SXM is an effective and safe drug for aplastic anemia. Its mechanism might be likely due to its regulating the immune function, which facilitates the recovery of the bone marrow hematopoiesis function. Content Type Journal ArticleCategory Original ArticlesDOI 10.1007/BF03178486Authors Yong-ming Zhou, Shanghai University of TCM Department of Hematopathy, Yueyang Hospital of Integrative Traditional Chinese and Western Medicine No. 110, Ganhe Road, Shanghai 200437 ShanghaiZhen-qiao Huang, Shanghai University of TCM Department of Hematopathy, Yueyang Hospital of Integrative Traditional Chinese and Western Medicine No. 110, Ganhe Road, Shanghai 200437 ShanghaiTao Huang, Shanghai University of TCM Department of Hematopathy, Yueyang Hospital of Integrative Traditional Chinese a...

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[Allogeneic cord blood transplantation for adults: current results and future development.]

Bulletin du Cancer — Fri, 18 Dec 2009 00:00:00 +0100

Authors: Labussiere H, Peffault de Latour R, Bay JO Hematopoietic stem cell transplantation is the choice treatment of many hematopoietic disorders. However, there is still no related or HLA-matched unrelated donor for one-third of the patients. Cord blood, which contains a lot of hematopoietic progenitors immunologicaly naive, represents not only an interesting alternative as hematopoietic stem cell source but also allows more HLA incompatibilities than the other sources. Promising results in children lead to develop cord blood transplantation in adults first of all in hematopoietic malignancies (acute leukemias and lymphoid diseases) and in a second time, in non-malignant diseases such as aplastic anaemia. The main problems for the development of this new strategy in adults are the p...

A Hospital-based Case Control Study of Aplastic Anemia in Shanghai, China.

Chemico-Biological Interactions — Fri, 18 Dec 2009 00:00:00 +0100

We report results of a hospital-based case control study of 137 consecutive patients diagnosed with aplastic anemia (AA) in participating hospitals over a 4 year period. Diagnoses were made by a single laboratory, subjects were age- and gender- matched to two controls and interviewed concerning previous disease, work histories and exposures to potential etiologic agents. Analysis was conducted on two distinct subgroups: severe aplastic anemia (SAA) and moderate aplastic anemia (MAA). In univariate regression models, the strongest associations were observed for exposure to benzene and SAA (OR=3.12, 95% CI=1.12-8.65) and life on a farm and MAA (OR=3.08, 95% CI=1.44-6.56). Benzene exposure did not show a strong dose-response relationship with either subtype. When accounting for all of the pot...

Behcet’s disease associated with malignancy in Korea: a single center experience

Rheumatology International — Thu, 17 Dec 2009 07:02:52 +0100

Abstract The aim of this study was to determine the clinical characteristics of Behcet’s disease (BD) complicated with malignancy in Korea. Of 1,769 patients with BD in our hospital, 32 patients (1.8%, 21 in solid cancer, 11 in hematologic malignancy) developed cancer. In 10 of the 32 subjects (31.3%), malignancy was diagnosed before or concomitantly with BD. Twelve cases (37.5%) occurred within the first 2 years of disease and 9 cases (28.1%) occurred 5 years after the diagnosis of BD. Myelodysplastic syndrome (MDS) was the most common disease (n = 7) followed by thyroid cancer (n = 4), breast cancer, cervix cancer, stomach cancer, rectal cancer, hepatoma, aplastic anemia (n = 3, each), renal cell cancer, endometrial cancer, lymphom...

Spontaneous Liver Rupture In A Patient With Peliosis Hepatis

Health News from Medical News Today — Thu, 10 Dec 2009 09:00:00 +0100

Peliosis hepatis (PH) is a rare pathological entity. PH varies from minimal asymptomatic lesions to larger massive lesions that may present with cholestasis, liver failure, portal hypertension, avascular mass lesion, or even spontaneous rupture. A research article published in the World Journal of Gastroenterology addresses this problem. The research team from Inha University School of Medicine reported a case of a 20-year-old male patient with aplastic anemia who presented with hemoperitoneum... (Source: Health News from Medical News Today)

Soliris(R) Reduced Hemolysis, Decreased Transfusion Requirements And Improved Fatigue In Patients With PNH And Bone Marrow Insufficiency Disorders

Health News from Medical News Today — Thu, 10 Dec 2009 09:00:00 +0100

Soliris® (eculizumab), a first-in-class terminal complement inhibitor developed by Alexion Pharmaceuticals, Inc. (Nasdaq: ALXN), reduced hemolysis (destruction of red blood cells) and transfusion requirements, and improved measures of fatigue, when added to ongoing immunosuppressive therapy (IST) in patients with both paroxysmal nocturnal hemoglobinuria (PNH) and bone marrow insufficiency (BMI), including aplastic anemia (AA)... (Source: Health News from Medical News Today)

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Hepatitis-associated aplastic anemia during a primary infection of genotype 1a torque teno virus

European Journal of Pediatrics — Wed, 09 Dec 2009 06:47:44 +0100

Abstract A 12-year-old Japanese boy suffered from severe acute hepatitis and pancytopenia. The patient underwent successful bone marrow transplantation from an HLA-identical sister. Torque teno virus (TTV) DNA of genotype 1a and IgM-class antibody against the virus were detected in sera at the onset of hepatitis. TTV/1a DNA and anti-TTV/1a IgM antibody levels were undetectable on the 16th and 46th days after the onset of illness, respectively. Anti-TTV/1a IgG antibody was positive throughout the observation period. Sequential viral load and anti-TTV/1a IgM antibody suggested a primary infection of TTV/1a. Genomic sequence of the virus coincided with that of the original strain first isolated from human. TTV DNA was quantified at 130 copies in 105 bone marrow mononuclear cel...

Spontaneous liver rupture in a patient with peliosis hepatis

EurekAlert! - Medicine and Health — Tue, 08 Dec 2009 05:00:00 +0100

(World Journal of Gastroenterology) A research team from South Korea presented a young male patient with aplastic anemia, who had received long-term treatment with oxymetholone. Their study suggest that peliosis hepatis be considered in patients with risk factors, with the possibility of potentially life-threatening complications. (Source: EurekAlert! - Medicine and Health)

Alemtuzumab is safe and effective as immunosuppressive treatment for aplastic anaemia and single-lineage marrow failure: a pilot study and a survey from the EBMT WPSAA

British Journal of Haematology — Mon, 07 Dec 2009 00:00:00 +0100

An alemtuzumab-based experimental immunosuppressive treatment (IST) regimen was investigated in 35 patients with severe aplastic anaemia (SAA), pure red cell (PRCA) or pure white cell aplasia (PWCA). Alemtuzumab total dose was 73[ndash]103 mg s.c., followed by cyclosporine. No serious toxicity due to the regimen was observed. Adverse events were clinically irrelevant; infectious events were rare. The total response rate was 58%, 84% and 100% in SAA, PRCA and PWCA, respectively, with corresponding 6 months cumulative response probabilities of 84%, 84% and 100%. Subcutaneous alemtuzumab is a feasible and sufficiently safe IST regimen for patients suffering from immune-mediated marrow failures. (Source: British Journal of Haematology)

Paroxysmal nocturnal haemoglobinuria clones are part of the immune dysregulation in hepatitis-associated aplastic anaemia

Alimentary Pharmacology and Therapeutics — Wed, 02 Dec 2009 00:00:00 +0100

(Source: Alimentary Pharmacology and Therapeutics)

Granulocyte transfusions in severe aplastic anemia: an eleven-year experience.

Haematologica — Tue, 01 Dec 2009 00:00:00 +0100

Conclusions Granulocyte transfusions may have an adjunctive role in severe infections in patients with severe aplastic anemia. HLA alloimmunization is not an absolute contraindication to granulocyte therapy. PMID: 19996117 [PubMed - in process] (Source: Haematologica)

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Immunophenotype of myeloid granulocytes: a pilot study for distinguishing myelodysplastic syndrome and aplastic anemia by flow cytometry

Clinical and Laboratory Haematology — Mon, 30 Nov 2009 00:00:00 +0100

It is often difficult to distinguish myelodysplastic syndrome (MDS) from aplastic anemia (AA) because of the considerable clinical, cytologic histologic similarities between these two disorders; however, distinguishing between AA and MDS is of great importance because there is a higher risk of progression to acute leukemia in patients with MDS compared with AA. Up to now, CD34+ cells in MDS and AA patients have been studied extensively; however, little information is available on myeloid granulocytes. The aim of this study was to determine whether immunophenotype of myeloid granulocytes in AA patients was different from that of MDS. Flow cytometry was used to assess the immunophenotype of myeloid granulocytes in 22 patients with MDS, 12 with AA, and 10 normal subjects. Our data showed that...

Tailoring iron chelation by iron intake and serum ferritin: prospective EPIC study of deferasirox in 1744 patients with transfusion-dependent anemias.

Haematologica — Mon, 30 Nov 2009 00:00:00 +0100

Conclusions Analysis of this large, prospectively collected data set confirms the response to chelation therapy across various anemias, supporting initial deferasirox doses based on transfusional iron intake, with subsequent dose titration guided by trends in serum ferritin and safety markers. PMID: 19951979 [PubMed - as supplied by publisher] (Source: Haematologica)

Asymptomatic diffuse "encephalitic" cerebral toxoplasmosis in a patient with chronic lymphocytic leukemia: case report and review of the literature.

International Journal of Clinical and Experimental Pathology — Thu, 19 Nov 2009 10:38:02 +0100

We describe a 70 year old male with stage IV chronic lymphocytic leukemia complicated by aplastic anemia. Neurological examination and imaging revealed no significant abnormalities. At autopsy, the brain revealed multifocal cysts and free tachyzoites of Toxoplasma gondii with diffuse microglial nodules and no necrosis. To the best of our knowledge, this case represents the first report of the "encephalitic" form of toxoplasmosis in a non-AIDS patient. PMID: 19918334 [PubMed - in process] (Source: International Journal of Clinical and Experimental Pathology)

Spontaneous liver rupture in a patient with peliosis hepatis: a case report.

World Journal of Gastroenterology : WJG — Thu, 19 Nov 2009 04:12:08 +0100

Authors: Choi SK, Jin JS, Cho SG, Choi SJ, Kim CS, Choe YM, Lee KY Peliosis hepatis is a rare pathological entity and may cause fatal hepatic hemorrhage and liver failure. Here, we present a young male patient with aplastic anemia, who had received long-term treatment with oxymetholone. The patient suffered from sudden onset of intra-abdominal hemorrhage with profuse hemoperitoneum. The patient was treated successfully with a right hemihepatectomy and is in good health after 13 postoperative months. We suggest that peliosis hepatis be considered in patients with hepatic parenchymal hematoma, especially in patients under prolonged synthetic anabolic steroid medication. The possibility of a potentially life-threatening complication of massive intra-abdominal bleeding should also be consi...

Deferasirox (Exjade) for the treatment of iron overload.

Acta Haematologica — Fri, 13 Nov 2009 15:22:05 +0100

Authors: Cappellini MD, Taher A Deferasirox is a once-daily oral iron chelator with established dose-dependent efficacy in both adult and pediatric patients with transfusional iron overload. The clinical development program has demonstrated the efficacy of deferasirox for up to 4.5 years of treatment in patients with various underlying anemias, including beta-thalassemia, myelodysplastic syndromes, sickle cell disease, aplastic anemia, and other rare anemias. In addition to reducing key indicators of total body iron levels (serum ferritin, liver iron concentration, and toxic labile plasma iron), deferasirox has also demonstrated the ability to remove cardiac iron and prevent future cardiac iron accumulation. Emerging long-term data confirm the tolerability profile of deferasirox, and d...

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Predictors of response to immunosuppressive therapy with antithymocyte globulin and cyclosporine and prognostic factors for survival in patients with severe aplastic anemia

European Journal of Haematology — Thu, 12 Nov 2009 00:00:00 +0100

Conclusions: Pre-IST neutrophil counts might predict the response to IST, while absolute ARCs prior to IST and response status after IST could be prognostic factors following IST. (Source: European Journal of Haematology)

A Pharmacovigilance Program From Laboratory Signals for the Detection and Reporting of Serious Adverse Drug Reactions in Hospitalized Patients.

Clinical Pharmacology and Therapeutics — Wed, 04 Nov 2009 00:00:00 +0100

We present the implementation of a prospective pharmacovigilance program based on automatic laboratory signals (ALSs) at a hospital. We also report the general findings after the first year of operation of the program, which involved ALSs that indicate various SADRs: agranulocytosis, aplastic anemia, liver injury, thrombocytopenia, hyponatremia, and rhabdomyolysis. The number of hospitalizations during the year was 54,525, and 1,732 patients experienced at least one ALS. The review of electronic medical records (EMRs) showed that no alternative cause (i.e., no non-SADR explanation) for the ALS was identified in 520 (30%) of the patients. After the individual ALS-patient evaluation, a total of 110 SADRs (6.35% of those identified after reviewing EMRs and 21.15% of those requiring individual...

Felbamate in an adult population with severe refractory epilepsy.

Ir Med J — Sun, 01 Nov 2009 00:00:00 +0100

Authors: Kearney H, Delanty N Felbamate (FBM) is efficacious in treating patients with refractory epilepsy but was withdrawn due to cases of aplastic anaemia, hepatic failure and five reported deaths. FBM is currently used in specialist centres and is only being used in one Irish centre to date. This papers aim is to review the efficacy and safety experience of FBM in Irish adult patients with refractory epilepsy. A retrospective chart review was done on patients' medical records. Patients were subdivided into responders and non responders based on change in seizure frequency and side effects were recorded for all. Of the 13 patients on FBM nine patients responded to FBM, four patients did not. FBM is a safe and efficacious alternative in an Irish adult population with refractory epile...

Comparison on In Vitro Characterization of Fucospheres and Chitosan Microspheres Encapsulated Plasmid DNA (pGM-CSF): Formulation Design and Release Characteristics.

AAPS PharmSciTech — Tue, 27 Oct 2009 00:00:00 +0100

In conclusion, the characterizations of the microspheres can be modulated by changing the formulation variables, and it can be concluded that fucospheres might be a potential carrier system for the controlled delivery of GM-CSF encoding plasmid DNA. PMID: 19859814 [PubMed - as supplied by publisher] (Source: AAPS PharmSciTech)

Temozolomide: Aplastic anaemia in an elderly patient: case report

Reactions — Mon, 26 Oct 2009 14:08:49 +0100

(Source: Reactions)

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The transplant iron score as a predictor of stem cell transplant survival

Journal of Hematology and Oncology — Fri, 23 Oct 2009 23:00:00 +0100

Recent studies have suggested that the presence of iron overload prior to stem cell transplantation is associated with decreased survival. Within these studies, the criteria used to define iron overload have varied considerably. Given the lack of consensus regarding the definition of iron overload in the transplant setting, we sought to methodically examine iron status among transplant patients. We studied 78 consecutive patients at risk for transfusion-related iron overload (diagnoses included AML, ALL, MDS, and aplastic anemia) who received either autologous or allogeneic stem cell transplant. Multiple measures of iron status were collected prior to transplantation and examined for their association with survival. Using this data, three potentially prognostic iron measures were identifie...

Chicken anemia virus infection in broiler chickens in Shahrekord, Iran: serological, hematological, and histopathological findings

Comparative Clinical Pathology — Wed, 14 Oct 2009 17:04:55 +0100

Abstract Chicken infectious anemia caused by a single-strand DNA circovirus is a disease in young chickens that is characterized by aplastic anemia, generalized lymphoid atrophy, and immunosuppression. In the present study, the presence of chicken anemia virus (CAV) infection and the hematologic and histopathologic changes in CAV seropositive broiler chickens in Shahrekord region, center of Iran, were investigated. Blood and lymphoid tissue samples were obtained from 271, 2–6-week-old chicks of 23 commercial broiler chicken flocks. Measurement of CAV antibody titers by enzyme-linked immunosorbent assay (ELISA) and differential leukocyte counts were carried out on serum and whole blood, respectively. The results of ELISA showed that all flocks of different ages were posit...

Hairy cell leukemia responsive to anti-thymocyte globulin used as immunosuppressive therapy for aplastic anemia.

International Journal of Hematology — Tue, 13 Oct 2009 23:00:00 +0100

Authors: Fujiwara S, Miyake H, Nosaka K, Yoshida M, Ishihara S, Horikawa K, Yonemura Y, Iyama K, Mitsuya H, Asou N Hairy cell leukemia (HCL) is occasionally misdiagnosed as aplastic anemia when only a few leukemic cells are present in the circulation. Here, we describe a patient with HCL who initially presented with pancytopenia and received a diagnosis of aplastic anemia. The patient was treated with immunosuppressive therapy including cyclosporine A and anti-thymocyte globulin (ATG). No blood cell transfusion was required for approximately 3 years after ATG therapy. She was referred to our hospital because of an abdominal mass and requiring periodic blood transfusions. A bone marrow biopsy at this time revealed proliferation of lymphocytes with a fried egg appearance and an increase ...

High-dose cyclophosphamide treatment for refractory severe aplastic anemia in children

Pediatric Blood and Cancer — Mon, 12 Oct 2009 23:00:00 +0100

To determine if high-dose cyclophosphamide is an effective therapy for children with refractory severe aplastic anemia (SAA).SAA is an illness characterized by the depletion of hematopoietic precursors associated with life-threatening complications. Hematopoietic stem cell transplant (HSCT) is the treatment of choice if a human leukocyte antigen (HLA)-related donor is available. Immunosuppression with anti-thymocyte globulin (ATG) and cyclosporine A (CSA) is an option for patients who are not HSCT candidates. Unrelated donor HSCT has been used with limited success. High-dose cyclophosphamide has been used successfully in the treatment of adults with SAA, but experience in children is limited.Five pediatric patients who had failed previous immunosuppressive therapy for SAA were treated with...

Varicella zoster virus vaccine live: Aplastic anaemia in an infant: case report

Reactions — Mon, 12 Oct 2009 13:02:30 +0100

(Source: Reactions)

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Implications of CD34+ cell dose on clinical and haematological outcome of allo-SCT for acquired aplastic anaemia

Bone Marrow Transplantation — Sun, 11 Oct 2009 23:00:00 +0100

Implications of CD34+ cell dose on clinical and haematological outcome of allo-SCT for acquired aplastic anaemia Bone Marrow Transplantation advance online publication, October 12, 2009. doi:10.1038/bmt.2009.267 Authors: M S Islam, P Anoop, P Datta-Nemdharry, D Sage, E C Gordon-Smith, D Turner, S Wiltshire, L O'Regan & J C W Marsh (Source: Bone Marrow Transplantation)

Association of HLA class II alleles with response to immunosuppressive therapy in Korean aplastic anemia patients.

Human Immunology — Wed, 07 Oct 2009 23:00:00 +0100

Authors: Song EY, Kang HJ, Shin HY, Ahn HS, Kim I, Yoon SS, Park S, Kim BK, Park MH HLA-DR15 (DR2) is overrepresented in aplastic anemia (AA) patients, and its presence is associated with a better response to cyclosporine-based immunosuppressive therapy (IST). However, little is known about other HLA alleles affecting therapy response. We investigated 37 Korean patients with severe AA for the association of HLA class II alleles with response to IST: cyclosporine A combined with anti-thymocyte globulin or anti-lymphocyte globulin. Molecular or serologic typing of HLA-DR and HLA-DQ alleles was performed. In responders (13/37, 35.1%), the frequency of HLA-DR15 was increased (69.2% vs 8.3%, p = 0.0002) and that of DR4 was decreased (7.7% vs 66.7%, p = 0.0007) than in non-responders. The re...

Nobel prize for medicine awarded for work on cancer

Guardian Unlimited Science — Mon, 05 Oct 2009 16:46:31 +0100

Discovery of how chromosomes are protected earns US trio top prize in medicine. Ian Sample reportsThe discovery of a biological gatekeeper that prevents genetic code from fraying with age has won a trio of American scientists this year's Nobel prize for medicine.The prestigious award – and the 10m Swedish kronor (£818,000) prize money – is shared by Elizabeth Blackburn, 60, Carol Greider, 48, and Jack Szostak, 56. It is the first time the prize has honoured two women at once.The researchers identified one of the most critical and intriguing processes in living organisms, one that has deep implications for understanding ageing, a variety of cancers and inherited diseases.Announcing the award at the Karolinska Institute in Stockholm, the Nobel assembly said the discoveries "added a new ...

Advances in the treatment of aplastic anemia

Revista Brasileira de Hematologia e Hemoterapia — Fri, 02 Oct 2009 16:48:26 +0100

Impressive response rates and the good tolerability have allowed imatinib to become the gold standard frontline therapy for all CML patients in the early chronic phase. Optimal outcomes are attained with more than two thirds of the CML cases treated with standard dose imatinib (400 mg daily). Criteria to establish failure and suboptimal responses to imatinib have been defined. Treatment guidelines have also suggested imatinib dose escalation based on clinical assessments of disease response. However, despite all the effort to optimize therapy with imatinib, cases of real resistance exist. For imatinib resistant and intolerant cases, second generation powerful tyrosine kinase inhibitors (TKIs) have been developed and registered. Sequential kinase inhibitor therapy is used to overcome resist...

Aplastic Anemia & MDS International Foundation Research Grant Opportunities

ScanGrants feed — Thu, 01 Oct 2009 23:00:00 +0100

Aplastic Anemia & MDS International Foundation Research Grant Opportunities AA&MDSIF is pleased to help researchers advance the understanding and treatment of aplastic anemia, myelodysplastic syndromes (MDS) and paroxysmal nocturnal hemoglobinuria (PNH). For more than twenty years, the AA&MDSIF has provided financial support to research that leads to new insights into the causes of bone marrow failure and the development of new therapeutic approaches.Each year the AA&MDSIF invites researchers to apply for two-year grants of $30,000 per year to test new ideas and to explore other research leads. The applications are ranked according to merit by a distinguished panel of research scientists and funded by the generosity of families and patients.Established investigators and new...

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Spectrum of anemia associated with chronic liver disease.

World Journal of Gastroenterology : WJG — Thu, 01 Oct 2009 22:20:37 +0100

Authors: Gonzalez-Casas R, Jones EA, Moreno-Otero R Anemia of diverse etiology is a common complication of chronic liver diseases. The causes of anemia include acute or chronic gastrointestinal hemorrhage, and hypersplenism secondary to portal hypertension. Severe hepatocellular disease predisposes to hemorrhage because of impaired blood coagulation caused by deficiency of blood coagulation factors synthesized by hepatocytes, and/or thrombocytopenia. Aplastic anemia, which is characterized by pancytopenia and hypocellular bone marrow, may follow the development of hepatitis. Its presentation includes progressive anemia and hemorrhagic manifestations. Hematological complications of combination therapy for chronic viral hepatitis include clinically significant anemia, secondary to treatm...

Systematic review - hepatitis-associated aplastic anaemia - a syndrome associated with abnormal immunological function

Gastroenterology and Liver Diseases Specialist Library - Liver — Thu, 01 Oct 2009 16:37:38 +0100

Published September 2009Bottom-line conclusion: Hepatitis-associated bone marrow aplasia is mediated by immunological mechanisms. Treatment options include hematopoietic cell transplantation and immunosuppressive therapy. (Source: Gastroenterology and Liver Diseases Specialist Library - Liver)

Thoracoscopic extended thymothymectomy for myasthenia gravis with aplastic anemia.

Annals of Thoracic and Cardiovascular Surgery — Thu, 01 Oct 2009 00:00:00 +0100

Authors: Nakamura H, Miwa K, Adachi Y, Fujioka S, Haruki T, Taniguchi Y It is very rare for both myasthenia gravis and aplastic anemia to be complicated with a thymoma. A 74-year-old female was diagnosed to have aplastic anemia with pancytopenia and systemic myasthenia gravis with severe restrictive respiratory dysfunction. Chest CT showed a 5-cm diameter thymoma. After platelets and packed red blood cells were transfused before surgery, an extended thymothymectomy was performed with a bilateral thoracoscopic approach. The thymoma was noninvasive, stage I, and was classified as B1 according to the World Health Organization classification. After the operation, the patient was managed on artificial ventilation with no complications. The myasthenia gravis remitted with the concomitant adm...

Estrogen-induced myelotoxicity in dogs: A review.

The Canadian Veterinary Journal — Thu, 01 Oct 2009 00:00:00 +0100

Authors: Sontas HB, Dokuzeylu B, Turna O, Ekici H Exogenous estrogens used for therapeutic purposes or endogenous estrogen sources such as functional Sertoli cell or ovarian granulosa cell tumors may cause bone marrow toxicity in dogs. The condition is characterized by hematologic abnormalities including thrombocytopenia, anemia, and leukocytosis or leukopenia. Despite intensive therapy with blood or platelet-rich transfusions, broad-spectrum antibiotics, steroids, and bone marrow stimulants, prognosis is unfavorable. Due to the the risk of stimulating the development of uterine diseases and the potential for inducing aplastic anemia, estrogen use in dogs is best avoided where possible. This paper describes the causes of estrogen-induced myelotoxicity, the clinical presentation of the ...

Transfusion related acute lung injury.

Indian Journal of Pathology and Microbiology — Wed, 30 Sep 2009 23:00:00 +0100

We describe a case of 10-year-old male child with aplastic anemia, platelet count of 7800/l, B positive blood group who developed fever (39.2C), difficulty in breathing and cyanosis within 2 hrs after transfusion of a random platelet concentrate. Despite the best resuscitative efforts, the child died within next 24 hrs. The present case highlights the fact that TRALI should be kept as a differential diagnosis in all patients developing acute respiratory discomfort within 6 hrs of transfusion. Without a 'gold standard' the diagnosis of TRALI relies on a high index of suspicion and on excluding other types of transfusion reactions. Notification to transfusion services is crucial to ensure that a proper investigation is carried out and at-risk donor and recipients can be identified, and risk ...

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Downregulation of GATA-2 and overexpression of adipogenic gene-PPARγ in mesenchymal stem cells from patients with aplastic anemia

Experimental Hematology — Wed, 23 Sep 2009 00:00:00 +0100

Aplastic anemia (AA) is characterized by a reduced number of hematopoietic stem cells and fatty replacement in the bone marrow. Transcriptional factor GATA-2 plays several important roles in both hematopoiesis and adipogenesis. Decreased levels of GATA-2 compromise the proliferation and survival of hematopoietic stem cells. GATA-2 suppresses adipocyte differentiation through direct inhibition of adipogenic factors, including peroxisome proliferator-activated receptor−γ (PPARγ). Previous studies have shown that expression of GATA-2 is decreased in marrow CD34-positive cells in AA. To elucidate the mechanisms of fatty marrow replacement, we evaluated the mRNA expression for GATA-2 and PPARγ in mesenchymal stem cells (MSCs) from patients with AA by quantitative real-time polymerase chain...

Ticlopidine: Aplastic anaemia: case report

Reactions — Mon, 21 Sep 2009 16:25:31 +0100

(Source: Reactions)

Use of a FLAER-based WBC assay in the primary screening of PNH clones.

American Journal of Clinical Pathology — Mon, 21 Sep 2009 05:12:22 +0100

Authors: Sutherland DR, Kuek N, Azcona-Olivera J, Anderson T, Acton E, Barth D, Keeney M Diagnosis of paroxysmal nocturnal hemoglobinuria (PNH) with flow cytometry traditionally involves the analysis of CD55 and CD59 on RBCs and neutrophils. However, the ability to accurately detect PNH RBCs is compromised by prior hemolysis and/or transfused RBCs. Patients with aplastic anemia (AA) and myelodysplastic syndrome (MDS) can also produce PNH clones. We recently described a multiparameter fluorescent aerolysin (FLAER)-based flow assay using CD45, CD33, and CD14 that accurately identified PNH monocyte and neutrophil clones in PNH, AA, and MDS. Here, we compared the efficiency of this WBC assay with a CD59-based assay on RBCs during a 3-year period. PNH clones were detected with the FLAER ass...

A Rare Case of Aplastic Anemia Caused by Temozolomide.

Southern Medical Journal — Tue, 15 Sep 2009 16:45:50 +0100

A 65-year-old female with glioblastoma multiforme (GBM) who developed aplastic anemia from temozolomide is reported. The patient presented with rapidly developing slurred speech, apathy, memory difficulties, an ataxic gait, and right-sided weakness. She was found to have GBM. She received standard external beam radiotherapy concomitant with temozolomide, followed by adjuvant temozolomide. On day 14 of adjuvant treatment, she presented with profound fatigue and spontaneous bruising and was noted to be severely pancytopenic. After an extensive workup, she was found to have aplastic anemia on bone marrow biopsy. This case represents a rare but potentially fatal toxicity from temozolomide.Page: 974DOI: 10.1097/SMJ.0b013e3181b1d2faAuthors: George, Benjamin J. MD; Eichinger, Jessica B. MD; Richa...

Tacrolimus/Methotrexate versus Cyclosporine/Methotrexate as Graft-versus-Host Disease Prophylaxis in Patients with Severe Aplastic Anemia Who Received Bone Marrow Transplantation from Unrelated Donors: Results of Matched Pair Analysis

Biology of Blood and Marrow Transplantation — Mon, 14 Sep 2009 00:00:00 +0100

Tacrolimus (FK) and cyclosporine (CsA) have been shown to be effective in the prophylaxis of graft-versus-host disease (GVHD). However, no comparative studies have yet been conducted to examine the efficacy of FK/methotrexate (MTX) and CsA/MTX in patients with severe aplastic anemia (SAA) given unrelated donor bone marrow transplantation (U-BMT). We used matched-pair analysis to compare FK/MTX with CsA/MTX in patients with SAA who received U-BMT through the Japan Marrow Donor Program. Forty-seven pairs could be matched exactly for recipient age and conditioning regimens. Forty-five patients achieved engraftment in the FK group and 42 patients in the CsA group. The probability of grade II-IV acute GVHD (aGVHD) was 28.9% in the FK group and 32.6% in the CsA group (P=.558). The probability of...

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Sex hormones, acting on the TERT gene, increase telomerase activity in human primary hematopoietic cells

Blood — Wed, 09 Sep 2009 23:00:00 +0100

Androgens have been used in the treatment of bone marrow failure syndromes without a clear understanding of their mechanism of action. Blood counts of patients with dyskeratosis congenita or aplastic anemia with mutations in telomerase genes can improve with androgen therapy. Here we observed that exposure in vitro of normal peripheral blood lymphocytes and human bone marrow–derived CD34+ cells to androgens increased telomerase activity, coincident with higher TERT mRNA levels. Cells from patients who were heterozygous for telomerase mutations had low baseline telomerase activity, which was restored to normal levels by exposure to androgens. Estradiol had an effect similar to androgens on TERT gene expression and telomerase enzymatic activity. Tamoxifen abolished the effects of both ...

Minor histocompatibility antigens on transfused leukoreduced units of red blood cells induce bone marrow transplant rejection in a mouse model

Blood — Wed, 09 Sep 2009 23:00:00 +0100

When successful, human leukocyte antigen (HLA)–matched bone marrow transplantation with reduced-intensity conditioning is a cure for several nonmalignant hematologic disorders that require chronic transfusion, such as sickle cell disease and aplastic anemia. However, there are unusually high bone marrow transplant (BMT) rejection rates in these patients. Rejection correlates with the number of transfusions before bone marrow transplantation, and it has been hypothesized that preimmunization to antigens on transfused blood may prime BMT rejection. Using a novel mouse model of red blood cell (RBC) transfusion and major histocompatibility complex–matched bone marrow transplantation, we report that transfusion of RBC products induced BMT rejection across minor histocompatibility an...

Improved outcome of patients older than 30 years receiving HLA-identical sibling hematopoietic stem cell transplantation for severe acquired aplastic anemia using fludarabine-based conditioning: a comparison with conventional conditioning regimen.

Haematologica — Mon, 31 Aug 2009 23:00:00 +0100

Authors: Maury S, Bacigalupo A, Anderlini P, Aljurf M, Marsh J, Socié G, Oneto R, Passweg JR, Older age is a limitation for HLA-identical sibling hematopoietic stem cell transplantation (HSCT) as first-line therapy for severe acquired idiopathic aplastic anemia (SAA). Fludarabine (Flu)-based conditioning might improve outcome in older patients. We analyzed retrospectively 30 patients older than 30 years receiving such reduced-intensity conditioning HSCT according to recommendations of the European Group for Blood and Marrow Transplantation (EBMT) and compared their outcome to a control group receiving the standard regimen (cyclophosphamide+/-antithymocyte globulin) over the same study period (1998-2007). Patients conditioned with Flu had a higher probability of overall survival t...

Incidence and risk factors of aplastic anemia in Latin American countries: the LATIN case-control study.

Haematologica — Mon, 31 Aug 2009 23:00:00 +0100

Conclusions The incidence of aplastic anemia in Latin America countries is low. Although the research study centers had a high coverage of health services, the underreporting of cases of aplastic anemia in selected regions can be discussed. Frequent exposure to benzene-based products increases the risk for aplastic anemia. Few associations with specific drugs were found, and it is likely that some of these were due to chance alone. PMID: 19734415 [PubMed - in process] (Source: Haematologica)

Feasibility and eligibility of retreatment with rabbit anti-T lymphocyte globulin for aplastic anemia previously treated with horse anti-thymocyte globulin.

International Journal of Hematology — Fri, 28 Aug 2009 23:00:00 +0100

Authors: Miura K, Hatta Y, Kobayashi S, Iriyama Y, Takei K, Takeuchi J PMID: 19728019 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)

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Subcutaneous alemtuzumab plus cyclosporine for the treatment of aplastic anemia

Annals of Hematology — Mon, 24 Aug 2009 16:51:32 +0100

Abstract Aplastic anemia (AA) is most frequently due to autoimmune attack on its own stem cells. Alemtuzumab is a monoclonal antibody which recognizes the CD52 antigen on the surface of T and B cells. It has proved useful in autoimmune diseases, lymphoproliferative conditions, and graft versus host disease. Based on its immunosuppressive properties, we treated 14 AA patients with alemtuzumab. Median age was 23 years. Ten milligrams of alemtuzumab were injected subcutaneously each day for five consecutive days. Cyclosporine A was also administered orally at a dose of 2 mg/kg every 12 h for 3 months, and then gradually tapered. Response to alemtuzumab was followed for a median of 20 months. There were eight responses (57.1%), two complete and six part...

Erosion of telomeric single-stranded overhang in patients with aplastic anaemia carrying telomerase complex mutations

European Journal of Clinical Investigation — Wed, 12 Aug 2009 23:00:00 +0100

Conclusions Telomeric overhang erosion does not participate in physiological ageing but support a role for eroded telomeric overhangs and abnormal telomere structure in pathological shortening of telomeres, especially caused by loss-of-function telomerase mutations. Disrupted telomere structure caused by short telomeric overhangs may contribute to the mechanisms of abnormal haematopoietic compartment senescence and chromosomal instability in human bone marrow failure. (Source: European Journal of Clinical Investigation)

Squamous cell carcinoma of base of tongue in a patient with Fanconi's anemia treated with radiation therapy: Case report and review of literature

Head and Neck — Mon, 10 Aug 2009 23:00:00 +0100

We report a case of Fanconi's anemia in a 27-year-old man with carcinoma of the base of tongue (T2N0M0) who was treated with radical radiation therapy to a dose of 70 Gy/35 fractions/51 days. We have also done in vitro radiosensitivity tests.The patient tolerated the radiation treatment well and completed it without any interruptions. In vitro studies did not show any increased radiosensitivity in this patient.Head and neck cancer in a patient with FA requires individualized treatment. The decision about opting for different modalities should be based on a balanced approach with respect to locoregional control and toxicities of the treatment. © 2009 Wiley Periodicals, Inc. Head Neck, 2009 (Source: Head and Neck)

Guidelines for the diagnosis and management of aplastic anaemia

British Journal of Haematology — Sun, 09 Aug 2009 23:00:00 +0100

(Source: British Journal of Haematology)

Salvage Allogeneic Hematopoietic Cell Transplantation with Fludarabine and Low-Dose Total Body Irradiation after Rejection of First Allografts

Biology of Blood and Marrow Transplantation — Sun, 02 Aug 2009 23:00:00 +0100

We summarized results in 38 consecutive patients (median age=56 years) with hematologic malignancies (n=35), aplastic anemia (n=2), or renal cell carcinoma (n=1), who underwent salvage hematopoietic cell transplantation (HCT) for allograft rejection. In 14 patients, the original donors were used for salvage HCT, and, in 24 cases, different donors were used. Conditioning for salvage HCT consisted of fludarabine (Flu) and either 3 or 4Gy total body irradiation (TBI). Sustained engraftment was achieved in 33 patients (87%). Grafts were rejected in 5 patients (13%), 4 of whom had myelofibrosis. With a median follow-up of 2 years (range: 0.3 to 7.8 years), the 2- and 4-year estimated survivals were 49% and 42%, respectively. The 2-year relapse rate and nonrelapse mortality (NRM) were 36% and 24...

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Successful salvage unrelated umbilical cord blood transplantation with two units after engraftment failure with single unit in severe aplastic anemia.

J Korean Med Sci — Fri, 31 Jul 2009 23:00:00 +0100

Authors: Lee JW, Kang HJ, Kim EK, Shin HY, Ahn HS Severe aplastic anemia (SAA) patients without an HLA-matched sibling donor need alternative treatment options. Umbilical cord blood transplantation (UCBT) has become an alternative means for treating various diseases, but it has not been proved to be a satisfactory method to treat SAA. Here, we report the case of a girl who underwent successful two-unit UCBT after engraftment failure with a single unit. Two-unit UCBT is proposed to have better engraftment potential and to offer a better chance of survival, according to some reports. Increased cell dose and graft-versus-graft reaction could contribute to these advantages. With this promising result, two-unit UCBT could be an alternative treatment option for patients with SAA without an H...

[Detection of Putative T cell Clones Using T cell Receptor beta Chain Gene Clonality Assay in Korean Patients with Aplastic Anemia.]

The Korean Journal of Laboratory Medicine — Fri, 31 Jul 2009 23:00:00 +0100

CONCLUSIONS: Putative pathogenetic T cell clones were detected in most of AA patients in the current study. T cell clonality assay would be useful for investigating the pathophysiology of acquired AA. PMID: 19726886 [PubMed - in process] (Source: The Korean Journal of Laboratory Medicine)

Understanding aplastic anaemia/bone-marrow failure syndromes

Paediatrics and Child Health — Fri, 31 Jul 2009 11:28:03 +0100

Abstract: The term ‘aplastic anaemias/bone-marrow failure syndromes’ encompasses a heterogeneous group of disorders, including rare inherited genetic diseases and ‘acquired’ idiopathic aplastic anaemia, all characterized by failure of haematopoiesis. In the majority of these conditions the natural history is progressive, and the clinical spectrum includes non-haematological manifestations and a predisposition to cancer. Recent advances have identified mutations in more than 30 different genes which can be grouped into five functional cellular pathways in inherited bone-marrow failure syndromes. Some of these genes have also been found to be mutated in ‘acquired’ idiopathic aplastic anaemia. Recent insight into the molecular pathophysiology of these disorders explains, at least ...

Origin and fate of blood cells deficient in glycosylphosphatidylinositol-anchored protein among patients with bone marrow failure

British Journal of Haematology — Mon, 27 Jul 2009 23:00:00 +0100

Peripheral blood from 489 recently diagnosed patients with aplastic anaemia (AA) and 316 with refractory anaemia (RA) of myelodysplastic syndrome was evaluated to characterize CD55[minus]CD59[minus] [paroxysmal nocturnal haemoglobinuria (PNH)]-type blood cells associated with bone marrow (BM) failure. PNH-type cells were detected in 57% and 20% of patients with AA and RA, respectively. The percentages of PNH-type granulocytes ranged from 0·003% to 94·2% and the distribution was log-normal with a median of 0·178%. Serial analyses of 75 patients with PNH-type cells over 5 years revealed that the percentage of PNH-type cells constantly increased in 13 (17%), persisted in 44 (59%), disappeared in the remaining 18 (24%) although even in the 'Disappearance' group, PNH-type granulocytes persis...

Successful first-line treatment with double umbilical cord blood transplantation in severe aplastic anemia

Bone Marrow Transplantation — Mon, 27 Jul 2009 23:00:00 +0100

Authors: H J Kang, J W Lee, H Kim, H Y Shin & H S Ahn (Source: Bone Marrow Transplantation)

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Allogeneic hematopoietic stem cell transplantation for acquired aplastic anemia using cyclophosphamide and antithymocyte globulin: a single center experience

Bone Marrow Transplantation — Sun, 26 Jul 2009 23:00:00 +0100

Authors: S Ladeb, A Abdelkefi, L Torjman, H Ben Neji, A Lakhal, H Kaabi, L Ben Hamed, S Ennigrou, S Hmida, T Ben Othman & A Ben Abdeladhim (Source: Bone Marrow Transplantation)

Anti-moesin antibodies derived from patients with aplastic anemia stimulate monocytic cells to secrete TNF-{alpha} through an ERK1/2-dependent pathway

International Immunology — Sun, 26 Jul 2009 23:00:00 +0100

Antibodies specific to moesin, which are frequently detectable in the serum of patients with aplastic anemia (AA), can induce tumor necrosis factor- (TNF-) secretion from monocytes and a human monocytic leukemia cell line THP-1. We investigated the mechanisms responsible for TNF- secretion from monocytic cells induced by the auto-antibodies that are purified from the sera of AA patients. TNF- induction by anti-moesin antibodies depended on the amount of cell surface moesin expressed by THP-1 cells. F(ab')2 fragments prepared from the anti-moesin antibodies were able to stimulate THP-1 cells to secrete TNF- and this stimulatory effect was enhanced by cross-linking of moesins with anti-human IgG F(ab')2 fragment antibodies. Anti-moesin antibodies as well as their F(ab')2 fragments induced th...

Allogeneic hematopoietic stem cell transplantation for acquired aplastic anemia using cyclophosphamide and antithymocyte globulin: a single center experience

Bone Marrow Transplantation — Sun, 26 Jul 2009 23:00:00 +0100

Authors: S Ladeb, A Abdelkefi, L Torjman, H Ben Neji, A Lakhal, H Kaabi, L Ben Hamed, S Ennigrou, S Hmida, T Ben Othman & A Ben Abdeladhim (Source: Bone Marrow Transplantation)

Thrombopoietin Agonists for the Treatment of Thrombocytopenia in Liver Disease and Hepatitis C

Clinics in Liver Disease — Thu, 23 Jul 2009 10:59:08 +0100

This article examines the nature of thrombocytopenia, ITP, and TPO. (Source: Clinics in Liver Disease)

Myelodysplastic syndromes.

American Journal of Clinical Pathology — Sat, 18 Jul 2009 18:44:13 +0100

Authors: Orazi A, Czader MB Session 4 of the 2007 Workshop of the Society for Hematopathology/European Association for Haematopathology was devoted to myelodysplastic syndromes (MDSs). Submitted cases highlighted important issues and difficulties in relation to the diagnosis and classification of MDS. Much of the discussion focused on the correlation, or lack of it, between morphologic examination and other diagnostic techniques, cytogenetics in particular. The cases included examples of isolated del(5q) chromosomal abnormality, including the "classical" 5q- syndrome and other myeloid neoplasms. Other cytogenetic abnormalities in MDSs and the role of cytogenetics in diagnosing MDSs were addressed. Particularly challenging is the correct identification of fibrotic subtypes of MDSs and t...

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Myocardial infarction caused by Aspergillus embolization in a patient with aplastic anemia

The Aspergillus Website - articles — Wed, 15 Jul 2009 23:00:00 +0100

Itoh M, Takahashi M, Mori M, Tamekiyo H, Yoshida H, Yago K, Shimada H, Arai K (Source: The Aspergillus Website - articles)

Changes of cytokine profile pre and post immunosuppression in acquired aplastic anemia.

Haematologica — Mon, 06 Jul 2009 23:00:00 +0100

This study suggests that IS does not fully clear excess TNF-alpha and IFN-gamma from marrow of patients with good outcome and raises the hypothesis that additional cytokine blockade might be useful in IS for AAA. PMID: 19586939 [PubMed - as supplied by publisher] (Source: Haematologica)

NKG2D-mediated immunity underlying paroxysmal nocturnal haemoglobinuria and related bone marrow failure syndromes

British Journal of Haematology — Sun, 05 Jul 2009 23:00:00 +0100

It is considered that a similar immune mechanism acts in the pathogenesis of bone marrow (BM) failure in paroxysmal nocturnal haemoglobinuria (PNH) and its related disorders, such as aplastic anaemia (AA) and myelodysplastic syndromes (MDS). However, the molecular events in immune-mediated marrow injury have not been elucidated. We recently reported an abnormal expression of stress-inducible NKG2D (natural-killer group 2, member D) ligands, such as ULBP (UL16-binding protein) and MICA/B (major histocompatibility complex class I chain-related molecules A/B), on granulocytes in some PNH patients and the granulocyte killing by autologous lymphocytes in vitro. The present study found that the expression of NKG2D ligands was common to both granulocytes and BM cells of patients with PNH, AA, and...

Patterns of infection in patients with aplastic anemia and the emergence of Aspergillus as a major cause of death

The Aspergillus Website - articles — Thu, 02 Jul 2009 23:00:00 +0100

Weinberger M, Elattar I, Marshall D, Steinberg SM, Redner RL, Young NS, Pizzo PA (Source: The Aspergillus Website - articles)

Bartonellosis as cause of death after red blood cell unit transfusion.

Ultrastructural Pathology — Tue, 30 Jun 2009 23:00:00 +0100

Authors: Magalhães RF, Urso Pitassi LH, Lania BG, Barjas-Castro ML, Neves Ferreira Velho PE The authors present the case of a young man with aplastic anemia who went into shock and died after several red blood cell unit transfusions. Immunohematological studies did not show any abnormality and blood cultures from patients and blood bags were negative. The ultrastructural findings, allied with current scientific knowledge, permitted the diagnosis of Bartonella sp. infection. In face of this diagnosis, two possibilities should be considered: the first one is that the patient was already infected by the bacteria before the last RBC unit transfusion. The pathogen could be involved in aplastic anemia etiology and in the failure to recover hemoglobin levels, in spite of the transfusions...

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Bacterial pneumonia following bone marrow transplantation: HRCT findings

Jornal Brasileiro de Pneumologia — Thu, 25 Jun 2009 08:23:17 +0100

CONCLUSIONS: The most common HRCT findings in our patient sample were air-space consolidation, small centrilobular nodules and ground-glass opacities, most often in the central and peripheral regions of the middle and lower lung zones.OBJETIVO: Descrever os achados de TCAR em pacientes com pneumonia bacteriana após transplante de medula óssea (TMO). MÉTODOS: Estudo retrospectivo com 30 pacientes diagnosticados com pneumonia bacteriana, documentada com TCAR do tórax realizada em até 24 h do início dos sintomas, e com diagnóstico comprovado com base em cultura positiva de escarro ou de aspirado brônquico associada à cultura positiva de líquido pleural ou de sangue dentro de uma semana após o início dos sintomas. Foram avaliados 20 pacientes masculinos e 10 femininos, com mediana ...

Advances and Challenges in Infectious Diseases Supportive Care of Patients With Hematologic Malignancies, Hematopoietic Stem Cell Transplantation, and Severe Aplastic Anemia

Seminars in Hematology — Thu, 25 Jun 2009 07:43:55 +0100

Infectious diseases are important causes of morbidity and mortality in immunocompromised patients with hematological malignancies, severe aplastic anemia (SAA), and myelodysplasia. Major advances in infectious diseases supportive care have been critical to improving the outcome of patients suffering from these life-threatening diseases. Advances in diagnosis, treatment, and prevention of life-threatening infections have reduced morbidity and mortality, improved quality of life, and enabled the use of potentially curative chemotherapy, radiation, hematopoietic stem cell transplantation (HSCT), and immunosuppressive therapy to patients battling these devastating diseases. Despite these advances, the continued development of antimicrobial resistance, emergence of new pathogens, and the evolut...

Antiviral Therapy in Patients With Hematologic Malignancies, Transplantation, and Aplastic Anemia

Seminars in Hematology — Thu, 25 Jun 2009 07:43:55 +0100

Advances in supportive care over the past two decades have decreased the morbidity and mortality attributed to opportunistic infections in immunocompromised patients, including those with hematologic malignancies, hematopoietic stem cell transplantation (HSCT), and aplastic anemia. Despite advances in antiviral therapy, opportunistic viral infections such as influenza, herpes simplex virus (HSV), varicella zoster virus (VZV), and cytomegalovirus (CMV) still cause significant morbidity and mortality in patients with compromised host defenses. Antiviral agents are key antimicrobials used for treatment and prophylaxis of viral infections in immunocompromised hosts. Currently, there are more than 40 antiviral agents approved for clinical use, but the majority of these agents are for the treatm...

Infections in Patients With Aplastic Anemia

Seminars in Hematology — Thu, 25 Jun 2009 07:43:55 +0100

Infection is a major cause of death in patients with aplastic anemia (AA). There are differences between the immunocompromised state of a patient with AA and the patient who is neutropenic due to chemotherapy and this leads to a difference in the infections that they incur. Prolonged neutropenia is one of the largest risk factors for the development of infections with the invasive mycoses and bacteria. Recovery from neutropenia is directly related to survival, and supportive care plays a large role in protection while the patient is in a neutropenic state. The most common invasive mycoses include the Aspergillus species, Zygomycetes, Candida spp., and Fusarium spp. Bacterial infections that are seen in patients with AA include gram-positive coagulase-negative Staphylococcus species, Entero...

Cancer in dyskeratosis congenita

Blood — Wed, 24 Jun 2009 23:00:00 +0100

Dyskeratosis congenita (DC) is a rare inherited bone marrow failure syndrome. The spectrum of cancer susceptibility in this disorder of telomere biology has not been described. There were more than 500 cases of DC reported in the literature from 1910 to 2008; the National Cancer Institute (NCI) prospective DC cohort enrolled 50 cases from 2002 to 2007. Sixty cancers were reported in 52 literature cases, while 7 occurred among patients in the NCI DC cohort. The 2 cohorts were comparable in their median overall survival (42 years) and cumulative incidence of cancer (40%-50% by age 50 years). The most frequent solid tumors were head and neck squamous cell carcinomas (40% of patients in either cohort), followed by skin and anorectal cancer. The ratio of observed to expected cancers (O/E ratio)...

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Systematic review: hepatitis-associated aplastic anaemia – a syndrome associated with abnormal immunological function

Alimentary Pharmacology and Therapeutics — Mon, 08 Jun 2009 23:00:00 +0100

Conclusions Hepatitis-associated bone marrow aplasia is mediated by immunological mechanisms. Treatment options include hematopoietic cell transplantation and immunosuppressive therapy. (Source: Alimentary Pharmacology and Therapeutics)

[Eosinophilic fasciitis and asplenia.]

Zeitschrift fur Rheumatologie — Thu, 04 Jun 2009 23:00:00 +0100

We report the case of a woman with eosinophilic fasciitis and congenital asplenia and discuss the possible causes and potential consequences. PMID: 19513726 [PubMed - as supplied by publisher] (Source: Zeitschrift fur Rheumatologie)

Mesalazine: Aplastic anaemia: case report

Reactions — Tue, 02 Jun 2009 22:36:40 +0100

(Source: Reactions)

New Study Results Verify That PNH Cells Are Found In Majority Of Patients With Bone Marrow Failure Syndromes

Health News from Medical News Today — Tue, 02 Jun 2009 07:00:00 +0100

Paroxysmal nocturnal hemoglobinuria (PNH) cells are present in the majority of patients with myelodysplastic syndromes (MDS), aplastic anemia (AA), and other bone marrow failure syndromes (BMF), according to interim results from 5,285 patients enrolled in the EXPLORE trial. (Source: Health News from Medical News Today)

Haematopoietic stem cell transplantation in Taiwan: past, present, and future.

Hong Kong Med J — Sun, 31 May 2009 23:00:00 +0100

In conclusion, HSCT has become a routine therapy for major medical centres in Taiwan. Our unique experiences in the past decades also contributed to the progress of HSCT. With the establishment of professional association and patient supportive groups, we hope we can fully improve our daily practice and clinical as well as basic research in HSCT. PMID: 19494390 [PubMed - in process] (Source: Hong Kong Med J)

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Mesalazine: Aplastic anaemia: case report.

Reactions Weekly — Sat, 30 May 2009 15:31:34 +0100

Page: 28 (Source: Reactions Weekly)

Antineutrophil cytoplasmic antibody associated vasculitis in one patient with severe aplastic anemia: Description of the first case

Leukemia Research — Tue, 19 May 2009 17:50:18 +0100

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides might present with joint symptoms and sinusitis. Rarely, classical destructive rheumatoid arthritis might be complicated by ANCA-associated microscopic polyangiitis or Wegener's granulomatosis . (Source: Leukemia Research)

Emesis predicts bacteremia in immunocompromised children with central venous catheters and fever

Cancer — Tue, 19 May 2009 04:00:00 +0100

The objective of this study was to determine whether vomiting at presentation of a febrile illness in immunocompromised children with central venous catheters (CVCs) predicts bacteremia.A chart review was conducted of children who were admitted to the hospital with a diagnosis of cancer or aplastic anemia, fever, and a CVC. Data were collected on the presence or absence of vomiting, catheter type, presence or absence of severe neutropenia, C-reactive protein (Crp) value, and culture results.There were 143 admissions for fever among 48 children. Among 35 admissions with emesis, 19 included bacteremia; whereas, among 107 admissions without emesis, 19 included bacteremia (P < .001). There was a 5-fold greater risk of bacteremia in children with children without vomiting (odds ratio, 5.50; 95%...

Aplastic anaemia in childhood. Description of two cases and review of the literature

Central European Journal of Medicine — Fri, 15 May 2009 06:02:54 +0100

We report two cases of aplastic anaemia having a different aetiology. The diagnostic work-up and the therapeutic management for each case are described below. Content Type Journal ArticleCategory Research ArticleDOI 10.2478/s11536-009-0024-7Authors Maria Scalzone, Catholic University of Rome Division of Paediatric Oncology 00168 Rome ItalyPaola Coccia, Catholic University of Rome Division of Paediatric Oncology 00168 Rome ItalyPalma Maurizi, Catholic University of Rome Division of Paediatric Oncology 00168 Rome ItalyGiorgio Attinà, Catholic University of Rome Division of Paediatric Oncology 00168 Rome ItalyLucia Liotti, Catholic University of Rome Division of Paediatric Oncology 00168 Rome ItalyMaria Giuseppina Cefalo, Catholic University of Rome Division of Paediatric Oncology 0016...

Coeliac disease and aplastic anaemia: a specific entity?

British Journal of Haematology — Tue, 05 May 2009 04:00:00 +0100

(Source: British Journal of Haematology)

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Aplastic anaemia: autoimmune sequel of thymoma

British Journal of Haematology — Tue, 05 May 2009 04:00:00 +0100

(Source: British Journal of Haematology)

Treatment of severe aplastic anemia by immunosuppressor anti-lymphocyte globulin/anti-thymus globulin as the chief medicine in combination with Chinese drugs

Chinese Journal of Integrative Medicine — Fri, 01 May 2009 06:37:49 +0100

Conclusions The therapeutic effect of combined therapy with Chinese drugs and ALG/ATG in treating SAA could be affirmed, showing some superiority as compared with Western medicine alone. The patients’ age, duration of illness, neutrophil count, and bone marrow proliferation before treatment, and degree of infection that occurred could affect the therapeutic efficacy to a certain extent. Adverse reactions resulting from the combined therapy are less, showing the toxicity reducing and effect enhancing action of Chinese drugs. Content Type Journal ArticleCategory Clinical ExperienceDOI 10.1007/s11655-009-0141-9Authors Bing-rong Zheng, First Clinical Medical College Affiliated to Zhejiang University of Traditional Chinese Medicine Department of Hematology Hangzhou 310...

Restoration of cardiac function by an iron chelator, deferasirox, in a patient with aplastic anemia and cardiac iron overload.

International Journal of Hematology — Thu, 30 Apr 2009 23:00:00 +0100

Authors: Kiguchi T, Ito Y, Kimura Y, Ohyashiki K PMID: 19363706 [PubMed - indexed for MEDLINE] (Source: International Journal of Hematology)

Identification and characterization of mutations in FANCL gene: A second case of Fanconi anemia belonging to FA-L complementation group

Human Mutation — Wed, 29 Apr 2009 04:00:00 +0100

In this study, we report an FA patient with an unusual presentation belonging to the FA-L complementation group. The patient lacks an obvious FA phenotype except for the presence of a café-au-lait spot, mild hypocellularity and a family history of leukemia. The molecular diagnosis and identification of the FA subgroup was achieved by FA complementation assay. We identified bi-allelic novel mutations in the FANCL gene and functionally characterized them. To the best of our knowledge, this is the second reported case belonging to the FA-L complementation group. © 2009 Wiley-Liss, Inc. (Source: Human Mutation)

Nephrogenic Diabetes Insipidus Induced by Two Amphotericin B Liposomal Formulations

Pharmacotherapy: Official Journal of the American College of Clinical Pharmacy — Mon, 27 Apr 2009 04:00:00 +0100

We describe an 18-year-old woman with aplastic anemia who developed invasive aspergillosis. She began treatment with high-dose (10 mg/kg/day) liposomal amphotericin B at home; however, her condition worsened, and she was hospitalized. Therapy with liposomal amphotericin B was continued until the patient began having symptoms consistent with nephrogenic DI. These symptoms resolved after discontinuation of liposomal amphotericin B; however, after rechallenge with lipid complex amphotericin B (5 mg/kg/day), the symptoms returned. The patient's nephrogenic DI was successfully treated with diuretics. Use of the Naranjo adverse drug reaction probability scale score indicated a probable relationship between liposomal amphotericin B and the development of nephrogenic DI. To our knowledge, this is ...

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Molecular 'Key' To Successful Blood Stem Cell Transplants Discovered

ScienceDaily Headlines — Sat, 25 Apr 2009 09:00:00 +0100

Researchers have discovered a "molecular key" that could help increase the success of blood stem cell transplants, a procedure currently used to treat diseases such as leukemia, Hodgkin's lymphoma and aplastic anemia. (Source: ScienceDaily Headlines)

Molecular "Key" To Successful Blood Stem Cell Transplants Discovered By UBC

Health News from Medical News Today — Thu, 23 Apr 2009 13:00:00 +0100

University of British Columbia researchers have discovered a "molecular key" that could help increase the success of blood stem cell transplants, a procedure currently used to treat diseases such as leukemia, Hodgkin's lymphoma and aplastic anemia. (Source: Health News from Medical News Today)

UBC research finds molecular "key" to successful blood stem cell transplants

EurekAlert! - Medicine and Health — Wed, 22 Apr 2009 04:00:00 +0100

(University of British Columbia) University of British Columbia researchers have discovered a "molecular key" that could help increase the success of blood stem cell transplants, a procedure currently used to treat diseases such as leukemia, Hodgkin's lymphoma and aplastic anemia. (Source: EurekAlert! - Medicine and Health)

Risk Factors Affecting Outcome of Second HLA-Matched Sibling Donor Transplantations for Graft Failure in Severe Acquired Aplastic Anemia

Biology of Blood and Marrow Transplantation — Mon, 20 Apr 2009 15:45:15 +0100

We examined transplantation outcomes after a second HLA-matched sibling transplantation for primary (16%) or secondary (84%) graft failure in 166 patients with severe acquired aplastic anemia (AA). Two-thirds of these patients has a performance score < 90. In most cases (88%), the same donor was used for both transplants, for both transplantations, and 84% of the second transplantations used bone marrow grafts. We identified 2 prognostic factors: intertransplantation interval (surrogate for primary graft failure and early secondary graft failure) and performance status. Shorter intertransplantation interval (≤ 3 months) and poor performance score (< 90) at second transplantation were associated with high mortality. In patients with a performance score of 90% to 100%, the 8-year probabili...

Multiple synchronous primary malignancies induced by benzene exposure: a case report

Journal of Occupational Medicine and Toxicology — Thu, 16 Apr 2009 04:00:00 +0100

Conclusion: This case reminds us that the occurrence of solid tumors should be monitored in workers with occupational history linked with a high concentration exposure to benzene, though it's rarely happened. (Source: Journal of Occupational Medicine and Toxicology)

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Periodontal management of a patient with severe aplastic anemia: a case report

Special Care in Dentistry — Wed, 15 Apr 2009 04:00:00 +0100

This article describes the periodontal management of a patient with severe aplastic anemia. A 51-year-old female patient was hospitalized with aplastic anemia and was referred for the management of her cyclosporine-induced gingival enlargement and bleeding from her gums. She also complained of pain in the palatal area and the mandibular anterior region. The patient was managed successfully with supra- and subgingival instrumentation, local drug delivery (LDD), electrosurgery, repeated oral hygiene instruction, and constant supervision. The treatment resulted in dramatic improvement in the patient's oral health and quality of life. This report highlights the importance of nonsurgical therapy followed by electrosurgery for the management of residual gingival enlargement. (Source: Special Car...

Antibacterials/ibuprofen/paracetamol: Aplastic anaemia in an elderly patient: case report

Reactions — Tue, 07 Apr 2009 16:41:49 +0100

(Source: Reactions)

Herbal medicines: Aplastic anaemia: case report

Reactions — Tue, 07 Apr 2009 16:41:49 +0100

(Source: Reactions)

Antibacterials/ibuprofen/paracetamol: Aplastic anaemia in an elderly patient: case report.

Reactions Weekly — Tue, 07 Apr 2009 16:32:50 +0100

Page: 6 (Source: Reactions Weekly)

Herbal medicines: Aplastic anaemia: case report.

Reactions Weekly — Tue, 07 Apr 2009 16:32:50 +0100

Page: 21 (Source: Reactions Weekly)

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Eosinophilic pustular folliculitis occurring after bone marrow transplantation in a child with aplastic anaemia.

Acta Derm Venereol A... — Tue, 07 Apr 2009 13:28:56 +0100

Authors: Ogawa E, Okuyama R, Niizuma H, Tagami H, Tsuchiya S, Aiba S PMID: 19326017 [PubMed - in process] (Source: Acta Derm Venereol A...)

Natural history of transfusion-independent non-severe aplastic anemia in children.

International Journal of Hematology — Fri, 03 Apr 2009 04:00:00 +0100

In conclusion, because the incidence of disease progression was high in patients with NSAA, a prospective randomized trial of early intervention with IST or observation alone until disease progression to SAA, followed by IST when the patients become transfusion-dependent is warranted. PMID: 19343478 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)

Dyskeratosis congenita.

Hematology/Oncology Clinics of North America — Wed, 01 Apr 2009 04:00:00 +0100

Authors: Savage SA, Alter BP Dyskeratosis congenita (DC) is an inherited bone marrow failure syndrome characterized clinically by the triad of abnormal nails, reticular skin pigmentation, and oral leukoplakia, and is associated with high risk of developing aplastic anemia, myelodysplastic syndrome, leukemia, and solid tumors. Patients have very short germline telomeres, and approximately half have mutations in one of six genes encoding proteins that maintain telomere function. Accurate diagnosis of DC is critical to ensure proper clinical management, because patients who have DC and bone marrow failure do not respond to immunosuppressive therapy and may have increased morbidity and mortality associated with hematopoietic stem cell transplantation. PMID: 19327580 [PubMed - in proces...

Acquired aplastic anemia in childhood.

Hematology/Oncology Clinics of North America — Wed, 01 Apr 2009 04:00:00 +0100

Authors: Guinan EC In comparison to past decades, children who have acquired aplastic anemia (AA) enjoy excellent overall survival that reflects improvements in supportive care, more accurate exclusion of children who have alternate diagnoses, and advances in transplantation and immunosuppressive therapy (IST). Matched sibling-donor hematopoietic stem cell transplants (HSCT) routinely provide long-term survival in the range of 90%, and 75% of patients respond to IST. In this latter group, the barriers to overall and complication-free survival include recurrence of AA, clonal evolution with transformation to myelodysplasia/acute myelogenous leukemia, and therapy-related toxicities. Improvements in predicting responses to IST, in alternative-donor HSCT, and in rationalizing therapy by un...

Diagnosis and treatment of acquired aplastic anemia.

Hematology/Oncology Clinics of North America — Wed, 01 Apr 2009 04:00:00 +0100

This article reviews the key diagnostic and prognostic factors that influence the choice of therapy in patients with acquired aplastic anemia. PMID: 19327577 [PubMed - in process] (Source: Hematology/Oncology Clinics of North America)

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Discordant outcomes in a case of parvovirus b19 transmission into both dichorionic twins.

Twin Research and Human Genetics — Wed, 01 Apr 2009 04:00:00 +0100

We present a dichorionic, diamniotic, dizygotic twin pregnancy after in vitro fertilization with parvovirus B19 infection and viral transmission to both twins, but different outcomes. At 19 weeks gestation, hydrops fetalis was diagnosed for male twin A, female twin B did not show any abnormalities. At 22 weeks gestation an acute parvovirus B19 infection was detected and twin A was diagnosed with intrauterine fetal death (IUFD) by ultrasound at 23 weeks gestation. Viral DNA was detected in maternal blood as well as in placenta and liver tissue of this twin. Twin B was born at 35 weeks gestation asymptomatically and no signs of hydrops or other congenital anomalies but viral DNA was detected by PCR in serum. At the age of 2 years, both IgG titres against B19 and parvovirus DNA amplification ...

Diagnosis and Treatment of Acquired Aplastic Anemia

Hematology/Oncology Clinics of North America — Wed, 01 Apr 2009 04:00:00 +0100

This article reviews the key diagnostic and prognostic factors that influence the choice of therapy in patients with acquired aplastic anemia. (Source: Hematology/Oncology Clinics of North America)

Acquired Aplastic Anemia in Childhood

Hematology/Oncology Clinics of North America — Wed, 01 Apr 2009 04:00:00 +0100

In comparison to past decades, children who have acquired aplastic anemia (AA) enjoy excellent overall survival that reflects improvements in supportive care, more accurate exclusion of children who have alternate diagnoses, and advances in transplantation and immunosuppressive therapy (IST). Matched sibling-donor hematopoietic stem cell transplants (HSCT) routinely provide long-term survival in the range of 90%, and 75% of patients respond to IST. In this latter group, the barriers to overall and complication-free survival include recurrence of AA, clonal evolution with transformation to myelodysplasia/acute myelogenous leukemia, and therapy-related toxicities. Improvements in predicting responses to IST, in alternative-donor HSCT, and in rationalizing therapy by understanding the pathoph...

Dyskeratosis Congenita

Hematology/Oncology Clinics of North America — Wed, 01 Apr 2009 04:00:00 +0100

Dyskeratosis congenita (DC) is an inherited bone marrow failure syndrome characterized clinically by the triad of abnormal nails, reticular skin pigmentation, and oral leukoplakia, and is associated with high risk of developing aplastic anemia, myelodysplastic syndrome, leukemia, and solid tumors. Patients have very short germline telomeres, and approximately half have mutations in one of six genes encoding proteins that maintain telomere function. Accurate diagnosis of DC is critical to ensure proper clinical management, because patients who have DC and bone marrow failure do not respond to immunosuppressive therapy and may have increased morbidity and mortality associated with hematopoietic stem cell transplantation. (Source: Hematology/Oncology Clinics of North America)

Bleeding polypoid lesions in the colon as a presentation of neutropenic colitis in aplastic anemia

Gastrointestinal Endoscopy — Wed, 01 Apr 2009 04:00:00 +0100

A 30-year-old man with a history of transfusion–dependent anemia presented with 2 days of rectal bleeding. Physical examination revealed vague tenderness in the lower quadrants of the abdomen. Complete blood count showed a white blood count of 2000/mm3 (normal 4000-10,000/mm3), a hemoglobin level of 7.1 g/dL (13.0-18.0 g/dL), and a platelet concentration of 14,000/mm3 (150,000-400,000/mm3). On colonoscopy, bleeding was found from multiple polypoid and fungiform lesions in the rectum (A) and cecum (B). Lesions were variable in size, ranging from 0.5 to 1.5 cm, friable, and covered with exudate. Some had superficial ulcerations from which blood oozed. CT scan of the abdomen showed circumferential and diffuse colonic bowel wall thickening in a layered pattern in the rectum and cecum (C). Hi...

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Aplastic anaemia and bone marrow failure

Medicine — Wed, 01 Apr 2009 04:00:00 +0100

Abstract: Aplastic anaemia (AA) is the main syndrome of bone marrow failure. The term implies failure of production of all three cell lineages with replacement of normal haemopoietic marrow by fat cells. The condition may be inherited or acquired. There is overlap with other causes of bone marrow failure such as hypoplastic myelodysplastic syndrome (MDS). Haemopoietic stem cells are reduced in number and proliferative potential. Emergence of abnormal cell clones is common, including paroxysmal nocturnal haemoglobinuria, MDS and acute myeloid leukaemia. Diagnosis is made by examination of peripheral blood and bone marrow. The blood shows pancytopenia. Remaining cells appear normal morphologically, as do cells in the fatty bone marrow aspirate. A bone marrow trephine is necessary to assess c...

Hematopoietic growth factors in aplastic anemia patients treated with immunosuppressive therapy-systematic review and meta-analysis.

Haematologica — Tue, 31 Mar 2009 04:00:00 +0100

Authors: Gurion R, Gafter-Gvili A, Paul M, Vidal L, Ben-Bassat I, Yeshurun M, Shpilberg O, Raanani P Immunosuppressive therapy is the treatment for aplastic anemia patients ineligible for transplantation. The role of hematopoietic growth factors as adjunct to treatment in these patients is unclear. We conducted a systematic review and meta-analysis of randomized controlled trials comparing treatment with immunosuppressive therapy and hematopoietic growth factors to immunosuppressive therapy alone in patients with aplastic anemia. Two reviewers appraised the quality of trials and extracted data. For each trial, results were expressed as relative risks with 95% confidence intervals (CI) for dichotomous data. The addition of hematopoietic growth factors yielded no difference in overall mo...

Aplastic anemia

MayoClinic.com Full Feed — Fri, 27 Mar 2009 05:00:00 +0100

— Comprehensive overview covers symptoms, causes, treatment of this rare blood disorder. Sponsored by:Chemotherapy.com - http://www.chemotherapy.com (Source: MayoClinic.com Full Feed)

Benzene activates caspase-4 and -12 at the transcription level, without an association with apoptosis, in mouse bone marrow cells lacking the p53 gene.

Archives of Toxicology — Fri, 27 Mar 2009 04:00:00 +0100

In conclusion, caspase-4 and -12 can be activated by benzene treatment without inducing cell apoptosis in mouse bone marrow, which are partly under the regulation of the p53 gene. PMID: 19326098 [PubMed - as supplied by publisher] (Source: Archives of Toxicology)

Lymphoproliferative Disorders after Immunosuppressive Therapy for Aplastic Anemia: A Case Report and Literature Review.

Acta Haematologica — Wed, 18 Mar 2009 04:00:00 +0100

We report DLBCL after immunosuppressive therapy for AA. Although this is a rare complication, it should be considered before beginning immunosuppressive therapy. PMID: 19295190 [PubMed - as supplied by publisher] (Source: Acta Haematologica)

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Allopurinol: Aplastic anaemia: case report

Reactions — Wed, 11 Mar 2009 13:52:59 +0100

(Source: Reactions)

Lenalidomide: Aplastic anaemia: case report

Reactions — Wed, 11 Mar 2009 13:52:59 +0100

(Source: Reactions)

Lymphocytes with aberrant expression of fas or fas ligand attenuate immune bone marrow failure in a mouse model.

Journal of Immunology — Sun, 08 Mar 2009 17:24:13 +0100

We examined cellular and functional characteristics of lpr and gld mutants on the C57BL/6 (B6) background. Lymph node (LN) cells from lpr and gld mice produced less apoptosis when coincubated with C.B10-H2(b)/LilMcd (C.B10) BM cells in vitro. This functional difference was confirmed by infusing lpr, gld, and B6 LN cells into sublethally irradiated CB10 mice. All donor LN cells showed significant T cell expansion and activation, but only B6 LN cells caused severe BM destruction. Mice infused with gld LN cells developed mild to moderate BM failure despite receiving FasL-deficient effectors, thus suggesting the existence of alternative pathways or incomplete penetrance of the mutation. Paradoxically, mice that received Fas-deficient lpr LN cells also had reduced BM failure, likely due to down...

Allopurinol: Aplastic anaemia: case report.

Reactions Weekly — Sat, 07 Mar 2009 03:20:47 +0100

Page: 5 (Source: Reactions Weekly)

Lenalidomide: Aplastic anaemia: case report.

Reactions Weekly — Sat, 07 Mar 2009 03:20:47 +0100

Page: 33 (Source: Reactions Weekly)

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Two cases of hepatocellular adenomatosis treated with transcatheter arterial embolization

Hepatology International — Thu, 05 Mar 2009 12:30:04 +0100

Abstract Hepatocellular adenoma is a rare benign tumor of the liver. However, some complications, such as hemorrhage, rupture, and malignant transformation, have been reported previously. Surgical resection is considered to be the best choice of treatment, when adenomas are increasing in size, while resection is difficult to perform when multiple adenomas develop throughout the liver. Here, we report two cases of multiple hepatocellular adenomatosis. One patient had a history of aplastic anemia and the other had glycogen storage disease. We treated them with transcatheter arterial embolization (TAE) to prevent hemorrhage and rupture. After TAE, most parts of the adenomas showed necrotic change. These cases suggest that TAE is an effective treatment of hepatocellular adenom...

MRI findings in aplastic anemia

American Journal of Hematology — Tue, 03 Mar 2009 00:00:00 +0100

No abstract. (Source: American Journal of Hematology)

Private Cord Blood Banking: Experiences and Views of Pediatric Hematopoietic Cell Transplantation Physicians

PEDIATRICS — Mon, 02 Mar 2009 05:00:00 +0100

CONCLUSIONS. Few transplants have been performed by using cord blood stored in the absence of a known indication in the recipient. Willingness to use banked autologous cord blood varies depending on disease and availability of alternative stem cell sources. Few pediatric hematopoietic cell transplantation physicians endorse private cord blood banking in the absence of an identified recipient, even for mixed-ethnicity children for whom finding a suitably matched unrelated donor may be difficult. (Source: PEDIATRICS)

Immunosuppressive treatment for aplastic anemia: are we hitting the ceiling?

Haematologica — Sun, 01 Mar 2009 05:00:00 +0100

Authors: Passweg JR, Tichelli A PMID: 19252172 [PubMed - in process] (Source: Haematologica)

Cost analysis of iron-related complications in a single institute.

The Korean Journal of Internal Medicine — Sun, 01 Mar 2009 05:00:00 +0100

CONCLUSIONS: The medical costs of IRCs are considerable, and more effective iron-chelating therapy is necessary to save medical resources and improve patient care. More in the way of comprehensive health and economic studies of IRCs are needed to allow both clinicians and health-policy makers to make better decisions. PMID: 19270479 [PubMed - in process] (Source: The Korean Journal of Internal Medicine)

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A retrospective compariso of cyclophosphamide plus antithymocyte globulin with cyclophosphamide plus busulfan as the conditioning regimen for severe aplastic anemia.

Braz J Med Biol Res — Sun, 01 Mar 2009 05:00:00 +0100

Authors: Ommati LV, Rodrigues CA, Silva AR, Silva LP, Chaufaille ML, Oliveira JS Allogeneic hematopoietic stem cell transplantation (AHSCT) is the treatment of choice for young patients with severe aplastic anemia (SAA). The association of antithymocyte globulin (ATG) and cyclophosphamide (CY) is the most frequently used conditioning regimen for this disease. We performed this retrospective study in order to compare the outcomes of HLA-matched sibling donor AHSCT in 41 patients with SAA receiving cyclophosphamide plus ATG (ATG-CY, N = 17) or cyclophosphamide plus busulfan (BU-CY, N = 24). The substitution of BU for ATG was motivated by the high cost of ATG. There were no differences in the clinical features between the two groups, including age, gender, cytomegalovirus status, ABO matc...

Dengue and aplastic anemia--a rare association.

Travel Medicine and Infectious Disease — Sun, 01 Mar 2009 05:00:00 +0100

Authors: Albuquerque PL, Silva Júnior GB, Diógenes SS, Silva HF Aplastic anemia is a medullary insufficiency secondary to the complete or partial disappearance of hematopoietic tissue without abnormal cellular proliferation. This is a rare complication of infections, such as dengue hemorrhagic fever. A 15-year-old girl was admitted with anemia, bleeding from the gums, petechiae and fever. Laboratory tests at the time of admission showed: Hemoglobin 4.8 g/dl; Hematocrit 13.4%; white blood count 2240/microl and platelets 11,500/microl. Dengue virus IgM antibodies were found. A bone marrow aspirate and biopsy showed severe aplastic anemia. Treatment with intravenous immunoglobulin and methylprednisolone was started. The patient had a favorable outcome. She was then given long-te...

Severe Allergic Reaction to Hydrochlorothiazide Mimicking Septic Shock

Pharmacotherapy: Official Journal of the American College of Clinical Pharmacy — Fri, 27 Feb 2009 05:00:00 +0100

We describe a patient who experienced a serious HCTZ-induced adverse event that, to our knowledge, has not yet been reported in the literature. A 78-year-old woman came to the emergency department with dyspnea and severe fatigue; her signs and symptoms were suggestive of septic shock from pneumonia. She was treated accordingly, her condition improved, and she was discharged home. During the next 2 months, the patient returned to the emergency department 2 more times and was hospitalized each time with the same diagnosis. During her third admission, it was discovered that the patient's primary care physician had restarted her HCTZ for hypertension after it had been discontinued during each of the first two hospitalizations. The patient's symptoms began within hours of the first and second h...

Dengue and aplastic anemia - A rare association.

Travel Medicine and Infectious Disease — Thu, 26 Feb 2009 20:29:05 +0100

Authors: Albuquerque PL, Silva Júnior GB, Diógenes SS, Silva HF Aplastic anemia is a medullary insufficiency secondary to the complete or partial disappearance of hematopoietic tissue without abnormal cellular proliferation. This is a rare complication of infections, such as dengue hemorrhagic fever. A 15-year-old girl was admitted with anemia, bleeding from the gums, petechiae and fever. Laboratory tests at the time of admission showed: Hemoglobin 4.8g/dl; Hematocrit 13.4%; white blood count 2240/mul and platelets 11,500/mul. Dengue virus IgM antibodies were found. A bone marrow aspirate and biopsy showed severe aplastic anemia. Treatment with intravenous immunoglobulin and methylprednisolone was started. The patient had a favorable outcome. She was then given long-term trea...

Phase II study of rabbit anti-thymocyte globulin, cyclosporine and granulocyte colony-stimulating factor in patients with aplastic anemia and myelodysplastic syndrome

Leukemia — Thu, 26 Feb 2009 12:33:30 +0100

Authors: R Garg, S Faderl, G Garcia-Manero, J Cortes, C Koller, X Huang, S York, S Pierce, M Brandt, M Beran, G Borthakur, H Kantarjian & F Ravandi (Source: Leukemia)

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ATG plus Cyclosporine Reduces All-Cause Mortality in Patients with Severe Aplastic Anemia - Systematic Review and Meta-Analysis.

Acta Haematologica — Wed, 25 Feb 2009 05:00:00 +0100

Conclusions: The combination of both drugs should be considered the gold standard only for patients with severe aplastic anemia. PMID: 19246887 [PubMed - as supplied by publisher] (Source: Acta Haematologica)

A survey of chloramphenicol use in plastic surgery: a follow-up

Journal of Plastic, Reconstructive & Aesthetic Surgery — Tue, 24 Feb 2009 00:00:00 +0100

Chloramphenicol ophthalmic ointment is a topical wound application commonly used following eye surgery, on facial suture lines and skin grafts. Our previous study reported widespread use of chloramphenicol ointment amongst UK plastic surgeons for prophylaxis, as topical treatment in established infection and as a moisturiser. Our recommendations at that time were to avoid its use purely as a moisturiser when suitable alternatives were available. In this way, the potential patient risk of serious allergic and other adverse drug reactions, such as aplastic anaemia, could be reduced. The current study set out to re-survey UK plastic surgeons to determine whether a change in practice has resulted on the basis of our previous recommendations. (Source: Journal of Plastic, Reconstructive & Ae...

Acquired immune mediated aplastic anemia: Is it antineoplastic?

Autoimmunity Reviews — Mon, 23 Feb 2009 05:00:00 +0100

Authors: Nissen C, Stern M There is increasing evidence that autoimmunity can inhibit growth of solid tumors. We propose that anti-tumor activity also operates in autoimmunity against hematopoietic stem cells in acquired aplastic anemia (AA). Reduction/dysfunction of regulatory T cells (T(REG)) in AA - rather than being the primary event - could be a response to insufficient or failing anti-tumor reactivity in predisposed individuals, causing elimination of tumor cells and collateral damage to adjacent normal hematopoietic tissue. This pathophysiological mechanism could also apply to otherwise unexplained pancytopenic syndromes which frequently occur in patients with leukemia and lymphoma and non-hematological malignancies. Observations supporting an anti-tumor effect of marrow hypopla...

Data Published In The Lancet Oncology Further Substantiates VIDAZA's Extended Survival Benefit For MDS Patients

Health News from Medical News Today — Sun, 22 Feb 2009 08:00:00 +0100

The Aplastic Anemia & MDS International Foundation says that data from the AZA-001 clinical trial, published in The Lancet Oncology, demonstrated that VIDAZA is the only non-transplant treatment that extends survival in higher-risk MDS patients. In the study, VIDAZA was also shown to prevent progression to acute myelogenous leukemia (AML) in patients with MDS. (Source: Health News from Medical News Today)