MedWorm: Aplastic Anemia

Antibodies in the Treatment of Aplastic Anemia

Archivum Immunologiae et Therapiae Experimentalis — Fri, 03 Feb 2012 17:10:31 +0100

Abstract Antibodies have been the cornerstone of treatment of acquired aplastic anemia for more than 25 years. Treatment with antithymocyte globulin (ATG) is considered pivotal and the addition of cyclosporine improves the overall response rate. This antibody is heterogeneous and horse ATG is apparently more effective than rabbit ATG. Several issues remain unsolved in relation to the combination of ATG and cyclosporine: cost, toxicity and late clonal disorders. In recent years, alternative immunosuppressive therapy has been proposed and new antibodies have emerged: porcine ATG, alemtuzumab, daclizumab, and rituximab. Experience with these antibodies is limited to a few studies with alemtuzumab being the most promising, but the results are interesting and provocative. ...

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Immunosuppressive Therapy in Aplastic Anemia

Indian Journal of Pediatrics — Tue, 24 Jan 2012 12:27:02 +0100

Conclusions The treatment of aplastic anemia in pediatric patients is a challenging task. One third of the patients achieved overall response which included both complete and partial response. Content Type Journal ArticleCategory Original ArticlePages 1-5DOI 10.1007/s12098-012-0691-2Authors Vineeta Gupta, Department of Pediatrics, Institute of Medical Sciences, Banaras Hindu University, Varanasi, 221005 IndiaAkash Kumar, Department of Pediatrics, Institute of Medical Sciences, Banaras Hindu University, Varanasi, 221005 IndiaVijai Tilak, Department of Pathology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, IndiaIsha Saini, Department of Pediatrics, Institute of Medical Sciences, Banaras Hindu University, Varanasi, 221005 IndiaBaldev Bhatia, Depar...

Antibodies to anti-thymocyte globulin in aplastic anemia patients have a negative impact on hematopoietic SCT

Bone Marrow Transplantation — Mon, 23 Jan 2012 05:00:00 +0100

Authors: C M Jol-van der Zijde, R G M Bredius, A M Jansen-Hoogendijk, F J Smiers, A C Lankester & M J D van Tol (Source: Bone Marrow Transplantation)

Karyotypic and fluorescent in-situ hybridization study of the centromere of chromosome 7 in secondary myeloid neoplasms

Revista Brasileira de Hematologia e Hemoterapia — Fri, 20 Jan 2012 22:10:14 +0100

CONCLUSION: This study enhances the importance of cytogenetic analysis of patients at the time of diagnosis of secondary myeloid neoplasms (Source: Revista Brasileira de Hematologia e Hemoterapia)

Thymoma followed by aplastic anemia: two different responses to immunosuppressive therapy

Revista Brasileira de Hematologia e Hemoterapia — Fri, 20 Jan 2012 22:10:14 +0100

Aplastic anemia is an uncommon complication of thymoma and is extremely infrequent after the surgical removal of a thymic tumor. Aplastic anemia is a result of marrow failure and is characterized by peripheral pancytopenia and severely depressed marrow cellularity; it may be an autoimmune manifestation of thymoma. As thymoma-associated hematological dyscrasias, which include pure red cell aplasia, aplastic anemia and myasthenia gravis, are supposed to be of immunologic origin, two cases of very severe aplastic anemia following the resection of lymphocytic thymomas treated with immunosuppression are herein presented (Source: Revista Brasileira de Hematologia e Hemoterapia)

Evaluation of concentration and storage effects of mitomycin C in the diagnosis of Fanconi anemia among idiopatic aplastic anemia patients

Indian Journal of Human Genetics — Fri, 20 Jan 2012 05:00:00 +0100

Conclusions: The study indicates that freshly made MMC stress test provides an unequivocal means of differentiation between FA and "idiopathic" aplastic anemia. Further, the study, the first of its kind from Iran, stresses on the need for conducting this test in all aplastic anemia cases, even those without congenital anomalies, for accurate and timely diagnosis of FA to implement appropriate therapy. (Source: Indian Journal of Human Genetics)

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Unrelated cord blood transplantation for newly diagnosed patients with severe acquired aplastic anemia using a reduced-intensity conditioning: high graft rejection, but good survival

Bone Marrow Transplantation — Mon, 16 Jan 2012 05:00:00 +0100

Authors: H-L Liu, Z-M Sun, L-Q Geng, X-B Wang, K-Y Ding, B-I Tang, J Tong & Z-Y Wang (Source: Bone Marrow Transplantation)

Activity of alemtuzumab monotherapy in treatment-naive, relapsed, and refractory severe acquired aplastic anemia

Blood — Thu, 12 Jan 2012 05:00:00 +0100

Antithymocyte globulin (ATG) + cyclosporine is effective in restoring hematopoiesis in severe aplastic anemia (SAA). We hypothesized that the humanized anti-CD52 mAb alemtuzumab might be active in SAA because of its lymphocytotoxic properties. We investigated alemtuzumab monotherapy from 2003-2010 in treatment-naive, relapsed, and refractory SAA in 3 separate research protocols at the National Institutes of Health. Primary outcome was hematologic response at 6 months. For refractory disease, patients were randomized between rabbit ATG + cyclosporine (n = 27) and alemtuzumab (n = 27); the response rate for alemtuzumab was 37% (95% confidence interval [CI], 18%-57%) and for rabbit ATG 33% (95% CI, 14%-52%; P = .78). The 3-year survival was 83% (95% CI, 68%-99%) for alemtuzumab and 60% (95% C...

Anti-AnWj causing acute hemolytic transfusion reactions in a patient with aplastic anemia.

Transfusion — Tue, 10 Jan 2012 05:00:00 +0100

CONCLUSION: A case of acute hemolytic transfusion reaction caused by anti-AnWj is reported in a patient with aplastic anemia requiring allogeneic stem cell transplantation. This is the second documented case of anti-AnWj to cause a hemolytic transfusion reaction. The case demonstrates the complexity of managing patients with rare antibodies and the importance of international collaboration in the management of these difficult cases. PMID: 22233405 [PubMed - as supplied by publisher] (Source: Transfusion)

New Topic Page: Aplastic Anemia

What's New on MedlinePlus — Thu, 05 Jan 2012 15:11:46 +0100

Visit the new MedlinePlus Health Topic page on Aplastic Anemia. Aplastic anemia is a rare but serious blood disorder. If you have it, your bone marrow doesn't make enough new blood cells. Causes include... (Source: What's New on MedlinePlus)

MedlinePlus: Aplastic Anemia

NLM General Announcements — Thu, 05 Jan 2012 06:23:17 +0100

(Source: NLM General Announcements)

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Health‐related quality of life (HRQL) in children with sickle cell disease and thalassemia following hematopoietic stem cell transplant (HSCT)

Pediatric Blood and Cancer — Mon, 19 Dec 2011 05:00:00 +0100

ConclusionsChildren with hemoglobinopathies had higher physical and emotional functioning scores prior to HSCT and experienced a similar pattern of recovery to their baseline functioning by 3 months post‐HSCT when compared to children receiving HSCT for acquired conditions. Pediatr Blood Cancer © 2011 Wiley Periodicals, Inc. (Source: Pediatric Blood and Cancer)

Exome sequencing identifies MPL as a causative gene in familial aplastic anemia.

Haematologica — Fri, 16 Dec 2011 05:00:00 +0100

This study shows for the first time a link between homozygous MPL mutations and familial aplastic anaemia. It also highlights the important role of MPL in trilineage haematopoiesis. PMID: 22180433 [PubMed - as supplied by publisher] (Source: Haematologica)

Long-term follow-up after allogeneic stem cell transplantation in patients with severe aplastic anemia after cyclophosphamide plus antithymocyte globulin conditioning.

Haematologica — Fri, 16 Dec 2011 05:00:00 +0100

Conclusions. Cyclophosphamide and antithymocyte globulin is an effective conditioning regimen for patients with severe aplastic anemia and is associated with low treatment-related mortality. Long-term complications include avascular necrosis and endocrine dysfunction.Key words: Hematopoietic stem cell transplantation, severe aplastic anemia, long-term outcomes. PMID: 22180425 [PubMed - as supplied by publisher] (Source: Haematologica)

Radioactivity by Marjorie C Malley – review

Guardian Unlimited Science — Thu, 15 Dec 2011 09:00:01 +0100

Discussion of radioactivity's cultural significance is divorced from the scientific narrative, which in turn is separated out according to theme in endless subdivisions. There's a sense of disjointedness, and the book ends up feeling too much like a course of study to fire the imagination. This is a missed opportunity: in a scientific world, stories of this kind should be as familiar to laypeople as Biblical narratives once were. Malley's writing, informative but sterile, fails to bring them alive.Science and natureDavid Shariatmadariguardian.co.uk © 2011 Guardian News and Media Limited or its affiliated companies. All rights reserved. | Use of this content is subject to our Terms & Conditions | More Feeds (Source: Guardian Unlimited Science)

Frequent loss of HLA alleles associated with copy number-neutral 6pLOH in acquired aplastic anemia

Blood — Thu, 15 Dec 2011 05:00:00 +0100

Idiopathic aplastic anemia (AA) is a common cause of acquired BM failure. Although autoimmunity to hematopoietic progenitors is thought to be responsible for its pathogenesis, little is known about the molecular basis of this autoimmunity. Here we show that a substantial proportion of AA patients harbor clonal hematopoiesis characterized by the presence of acquired copy number-neutral loss of heterozygosity (CNN-LOH) of the 6p arms (6pLOH). The 6pLOH commonly involved the HLA locus, leading to loss of one HLA haplotype. Loss of HLA-A expression from multiple lineages of leukocytes was confirmed by flow cytometry in all 6pLOH(+) cases. Surprisingly, the missing HLA-alleles in 6pLOH(+) clones were conspicuously biased to particular alleles, including HLA-A*02:01, A*02:06, A*31:01, and B*40:0...

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An Epstein-Barr Virus-Associated Leukemic Lymphoma in a Patient Treated with Rabbit Antithymocyte Globulin and Cyclosporine for Hepatitis-Associated Aplastic Anemia.

Acta Haematologica — Thu, 15 Dec 2011 05:00:00 +0100

Authors: Ohata K, Iwaki N, Kotani T, Kondo Y, Yamazaki H, Nakao S Abstract Lymphoproliferative disorders (LPDs) are generally caused by uncontrolled B-cell proliferation induced by the Epstein-Barr virus (EBV) in the setting of impaired EBV-specific T-cell immunity, particularly when there is pharmacological immunosuppression including antithymocyte globulin. We herein present an unusual case of EBV associated with LPD (EBV-LPD) in which LPD occurred 3 weeks after the use of rabbit antithymocyte globulin administered for severe hepatitis-associated aplastic anemia; the patient died of fulminant leukemic lymphoma 5 days after the onset. We also review the pertinent literature on EBV-LPD after immunosuppressive therapy and document the efficacy of EBV viral load monitoring and the ne...

Analysis of the prognostic factors of very severe aplastic anemia treated with Chinese Kidney-invigorating drugs in combination with anti-lymphocyte globulin or anti-thymocyte globulin

Chinese Journal of Integrative Medicine — Thu, 08 Dec 2011 18:25:39 +0100

Conclusions GSKD+ALG/AT6 therapy for treatment of VSAA could enhance the current clinical therapeutic effects and elevate patients’ survival rate. Conditions including male sex, age >20 years, illness course ⩽1 month, neutrophil count >0.1 × 109/L, and reticulocyte count >10 × 109/L are the likely effective indices for predicting favorable therapeutic effectiveness in newly diagnosed VSAA patients. Content Type Journal ArticleCategory Original ArticlePages 1-6DOI 10.1007/s11655-011-0874-0Authors Xu-dong Tang, Department of Hematology, Xiyuan Hospital China Academy of Chinese Medical Sciences, Beijing, 100091 ChinaFeng Liu, Department of Hematology, Xiyuan Hospital China Academy of Chinese Medical Sciences, Beijing, 100091 ChinaLiu Li, Department of Hem...

Third‐party mesenchymal stromal cell infusion is associated with a decrease in thrombotic microangiopathy symptoms observed post‐hematopoietic stem cell transplantation

Pediatric Transplantation — Wed, 07 Dec 2011 05:00:00 +0100

The genetics of dyskeratosis congenita

Cancer Genetics and Cytogenetics — Thu, 01 Dec 2011 05:00:00 +0100

Dyskeratosis congenita (DC) is an inherited bone marrow failure syndrome associated with characteristic mucocutaneous features and a variable series of other somatic abnormalities. The disease is heterogeneous at the genetic and clinical levels. Determination of the genetic basis of DC has established that the disease is caused by a number of genes, all of which encode products involved in telomere maintenance, either as part of telomerase or as part of the shelterin complex that caps and protects telomeres. There is overlap at the genetic and clinical levels with other, more common conditions, including aplastic anemia (AA), pulmonary fibrosis (PF), and liver cirrhosis. Although part of the spectrum of disorders known to be associated with DC, it has emerged that mutations in telomere mai...

[Comment] Rabbit ATG for aplastic anaemia treatment: a backward step?

LANCET — Fri, 25 Nov 2011 12:00:53 +0100

Advances in medical care and research invariably result in improved outcomes for patients over time. Aplastic anaemia is a rare but serious form of bone-marrow failure that results in severe pancytopenia, a high risk of life-threatening infection, and haemorrhage. Over the past five decades, survival of patients has improved from around 25% to 60–80%, as allogeneic bone-marrow transplantation or immunosuppressive therapy have become available. However, a recent change in the availability of immunosuppressive therapy drugs has had a negative impact on patients' outcome. (Source: LANCET)

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Renal mucormycosis in aplastic anemia: a novel presentation

International Urology and Nephrology — Tue, 22 Nov 2011 17:59:44 +0100

We describe the case of a 20-year-old man with such a rare association. Content Type Journal ArticleCategory Nephrology - Case ReportPages 1-4DOI 10.1007/s11255-011-0078-8Authors Ashish Khandelwal, University of Ottawa, Ottawa, ON, CanadaPreeti Gupta, CGHS, New Delhi, IndiaAnkur Gupta, University of Ottawa, Ottawa, ON, CanadaVivek Virmani, University of Ottawa, Ottawa, ON, Canada Journal International Urology and NephrologyOnline ISSN 1573-2584Print ISSN 0301-1623 (Source: International Urology and Nephrology)

The polymorphisms of T cell-specific TBX21 and STAT4 genes may contribute to the susceptibility of Chinese individuals to aplastic anemia.

Human Immunology — Sun, 20 Nov 2011 05:00:00 +0100

Authors: Ge M, Zheng Y, Li X, Shi J, Ge J, Li H, Feng S Abstract T cell-specific T-box transcription factor gene (TBX21) and signal transducer and activator of transcription 4 (STAT4) have been suggested as 2 candidate genes for conferring susceptibility to autoimmunity. We herein hypothesized that the polymorphisms of TBX21 and STAT4 genes might contribute to the susceptibility of Chinese individuals to aplastic anemia (AA) as T cell-mediated immune disease characterized by hypoplasia and pancytopenia. We investigated the distributions of TBX21 (T-1993C and T-1514C) and STAT4 (rs7574862) polymorphisms in 202 adult patients with AA and 195 healthy controls by polymerase chain reaction-restriction fragment length polymorphism. The frequencies of T-1993C (but not T-1514C) genotype an...

Congenital Amegakaryocytic Thrombocytopenia: Clinical Presentation, Diagnosis, and Treatment

Seminars in Thrombosis and Hemostasis — Fri, 18 Nov 2011 05:00:00 +0100

Semin Thromb Hemost 2011; 37: 673-681DOI: 10.1055/s-0031-1291377ABSTRACTCongenital amegakaryocytic thrombocytopenia (CAMT, MIM #604498) is a rare inherited bone marrow failure syndrome presenting as isolated hypomegakaryocytic thrombocytopenia at birth without other characteristic physical anomalies. Most of the patients develop a severe aplastic anemia and trilineage cytopenia during the first years of life and hematopoietic stem cell transplantation is the only curative treatment. In most of the cases the disease is caused by homozygous or compound heterozygous mutations in the gene MPL encoding the receptor for the hematopoietic growth factor thrombopoietin. The present review summarizes clinical and laboratory data for 96 patients with CAMT, reported since 1990.[...]© Thieme Medical P...

Allo-SCT in a patient with CRMCC with aplastic anemia using a reduced intensity conditioning regimen

Bone Marrow Transplantation — Mon, 14 Nov 2011 05:00:00 +0100

Authors: D Asai, S Osone, T Imamura, H Sakaguchi, N Nishio, H Kuroda, S Kojima & H Hosoi (Source: Bone Marrow Transplantation)

Tolerance to liver allograft after allogeneic hematopoietic cell transplantation for severe aplastic anemia from the same HLA-matched sibling donor

Bone Marrow Transplantation — Mon, 14 Nov 2011 05:00:00 +0100

Authors: H Sun, G Therapondos, J Lipton & V Gupta (Source: Bone Marrow Transplantation)

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Horse versus Rabbit Antithymocyte Globulin in Aplastic Anemia

New England Journal of Medicine — Wed, 09 Nov 2011 22:00:30 +0100

New England Journal of Medicine, Volume 365, Issue 19, Page 1842-1844, November 2011. (Source: New England Journal of Medicine)

Haploidentical haematopoietic stem cell transplantation using CD3 or CD3/CD19 depletion and conditioning with fludarabine, cyclophosphamide and antithymocyte globulin for acquired severe aplastic anaemia

British Journal of Haematology — Sat, 05 Nov 2011 04:00:00 +0100

(Source: British Journal of Haematology)

Telomere length is associated with disease severity and declines with age in dyskeratosis congenita.

Haematologica — Fri, 04 Nov 2011 04:00:00 +0100

Conclusions: Telomere length by flow fluorescence in situ hybridization is an important diagnostic test for dyskeratosis congenita; age-adjusted values provide a quantitative measure of disease severity (clinical subset, mutated gene, and degree of bone marrow failure). Patients with dyskeratosis congenita have accelerated telomere shortening.Keywords: bone marrow failure, dyskeratosis congenita, telomeres, longitudinal studyTrial registration: This study is registered at www.clinicaltrials.gov (identifier: NCT00027274). PMID: 22058220 [PubMed - as supplied by publisher] (Source: Haematologica)

Orofacial manifestations of hematological disorders: Anemia and hemostatic disorders

Table of Contents : Indian Journal of Dental Research : 2006 - 17(3) — Thu, 03 Nov 2011 04:00:00 +0100

Titilope A Adeyemo, Wasiu L Adeyemo, Adewumi Adediran, Abd Jaleel A Akinbami, Alani S AkanmuIndian Journal of Dental Research 2011 22(3):454-461The aim of this paper is to review the literature and identify orofacial manifestations of hematological diseases, with particular reference to anemias and disorders of hemostasis. A computerized literature search using MEDLINE was conducted for published articles on orofacial manifestations of hematological diseases, with emphasis on anemia. Mesh phrases used in the search were: oral diseases AND anaemia; orofacial diseases AND anaemia; orofacial lesions AND anaemia; orofacial manifestations AND disorders of haemostasis. The Boolean operator "AND" was used to combine and narrow the searches. Anemic disorders associated with orofacial sig...

Hemophagocytic lymphohistiocytosis after hematopoietic stem cell transplantation in children: A nationwide survey in Japan

Pediatric Blood and Cancer — Mon, 31 Oct 2011 00:04:55 +0100

ConclusionsThese findings suggest that early‐onset post‐HSCT HLH is a specific entity of HLH, and appropriate diagnosis and prompt management need to be established. Pediatr Blood Cancer © 2011 Wiley Periodicals, Inc. (Source: Pediatric Blood and Cancer)

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Survival After Immunosuppressive Therapy in Children with Aplastic Anemia.

Indian Pediatrics — Sun, 30 Oct 2011 04:00:00 +0100

CONCLUSIONS: The survival of patients who respond to IST is excellent. Also, G-CSF reduces the infectious complications without conferring any survival advantage. PMID: 22080620 [PubMed - as supplied by publisher] (Source: Indian Pediatrics)

Danazol-induced life-threatening cerebral venous thrombosis in a patient with aplastic anemia

Neurology India — Sat, 22 Oct 2011 04:00:00 +0100

G Sudheer Kumar, VR Roopesh Kumar, MS Gopalakrishnan, CV Shankar Ganesh, MS VenkateshNeurology India 2011 59(5):762-764 (Source: Neurology India)

The initial presentation of systemic lupus erythematosis with aplastic anemia successfully treated with rituximab

Clinical Rheumatology — Thu, 20 Oct 2011 15:52:31 +0100

We report a patient with the diagnosis of secondary aplastic anemia associated with lupus. All routine medications were not effective. She received Rituximab and her response was satisfactory. Her hematologic parameters were within normal range until last follow-up, six months after therapy was initiated with Rituximab. Review of literature displayed 23 cases of acquired aplastic anemia secondary to systemic lupus erythematosis; however, this is the first time the new drug therapy was used for the treatment. Content Type Journal ArticleCategory Case Based ReviewPages 1-4DOI 10.1007/s10067-011-1878-zAuthors Gholam-Hossein Alishiri, Department of Internal Medicine, Baqiyatallah University of Medical Sciences, Tehran, Islamic Republic of IranAmin Saburi, Health Research Center, Baq...

Short telomeres result in chromosomal instability in hematopoietic cells and precede malignant evolution in human aplastic anemia

Leukemia — Tue, 18 Oct 2011 04:00:00 +0100

Authors: R T Calado, J N Cooper, H M Padilla-Nash, E M Sloand, C O Wu, P Scheinberg, T Ried & N S Young (Source: Leukemia)

Association of Celiac Disease with Aplastic Anemia

Indian Journal of Pediatrics — Mon, 10 Oct 2011 15:01:12 +0100

Abstract The authors report a case of 13-y-old boy who presented with pain in bilateral knee and ankle joints accompanied by petechiae all over the body and hematochezia. On investigation, he had pancytopenia, which on subsequent evaluation revealed aplastic anemia. On investigation for short stature, he was diagnosed as a case of celiac disease. He was started on gluten free diet and the counts improved over 8 mo. Association between aplastic anemia and celiac disease has rarely been reported. To the best of authors’ knowledge only 8 adult cases of celiac disease associated with aplastic anemia have been published. This is the first report to suggest an association in children and response to a gluten free diet. Content Type Journal ArticleCategory Clinical Bri...

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Communication of benefits and risks of medical radiation: a historical perspective.

Health Physics — Sat, 08 Oct 2011 10:52:22 +0100

Authors: Timins JK Abstract X-rays were discovered by Wilhelm Röntgen in 1895. Within one year, benefits of x-rays, such as visualization of fractures, and detriments, such as x-ray dermatitis, were recognized. Nobel Laureates Pierre and Marie Sklodowska Curie discovered the radioactive element radium in 1898, and a year later the application of radiation to cure cancer was reported. A significant price was paid for this: Marie Curie died of aplastic anemia related to her radiation exposure, and her daughter Irene Joliot Curie, Nobelist for radiochemical research, died of radiation-induced leukemia. Internationally developed radiation protection recommendations were formalized starting in the late 1920s. The increasing use of ionizing radiation in medical diagnosis and radiation t...

Prognosis of acute invasive fungal rhinosinusitis related to underlying disease

International Journal of Infectious Diseases — Mon, 03 Oct 2011 04:00:00 +0100

Conclusions: Early medical and surgical treatment is essential to improve the prognosis of AIFRS patients. A poorer prognosis was associated with underlying disease and extensiveness of AIFRS, but not to the fungus isolated. (Source: International Journal of Infectious Diseases)

Aplastic anemia in association with a lymphoproliferative neoplasm: Coincidence or causality?

Leukemia Research — Mon, 03 Oct 2011 04:00:00 +0100

Aplastic anemia (AA) is defined as a pancytopenia associated with unexplained bone marrow hypocellularity. The incidence of acquired AA in the Western hemisphere is around 2 new cases per million inhabitants per year . Age distribution shows peaks in children and young adults and in patients aged >60 years. Patients with AA commonly present with anemia, skin or mucosal hemorrhages and, less frequently, with infection. Acquired AA can be considered in most cases as a T cell-mediated autoimmune disorder, targeted against the hematopoietic progenitors, leading to bone marrow failure . Viral infections, drugs, exposure to chemicals, pregnancy, or unknown agents seem to trigger autoimmunity in patients with predisposition. Associations of AA with other autoimmune diseases have been shown in a r...

Autologous transplant in the treatment of severe aplastic anemia – A case report

Transfusion and Apheresis Science — Sat, 01 Oct 2011 04:00:00 +0100

We report a 35year-old sAA male patient who initially underwent IST using rabbit ATG and Cyclosporine A (CsA). He was supportive transfusion dependent for the whole period of IST-phase. After the second IST-cycle, polymorphonuclear (PMN) cell count increase (>2.0×109/L) was observed, when SC mobilization, two large volume leukapheresis procedures and following autologous transplant were performed.The yields of harvested CD34+ and CD34+/CD90+ cells were 5.75×106/kgbm and 1.7×106/kgbm, respectively. The quantity of applied CD34+ and CD34+/CD90+ cells in autologous SC transplant were 5.45×106/kgbm (7-AADCD34+viability=95.42%) and 1.63×106/kgbm (7-AADCD34+/CD90+viability=95.42%), respectively. Hematopoietic reconstitution registered due to second month after autologous SC transplant and h...

Clinical specificities of 246 Japanese with prostate anomalies: early bone metastases, aplastic anemia, lymphatic-related lung lesions, primary pleural and lung cancers, and fatty liver with insulin-resistant diabetes, which were related to prostate fusion gene anomalies

Comparative Clinical Pathology — Mon, 19 Sep 2011 13:44:47 +0100

Abstract A total of 246 Japanese composed of 121 with benign prostatic hyperplasia (BPH) and 125 with prostatic cancer (PCa) were analyzed clinically to explain fusion gene anomalies between erythroblast transformation-specific family transcription factor-related gene (ERG) and transmembrane protease serine 2 (TMPRSS2) on chromosome 21q22. Among the 246 cases, 12 (10%) BPH and 18 (14%) PCa showed prostate-specific antigen (PSA) values of >20 ng/ml. It was shown that PSA was not only secreted from the prostates of BPH and PCa, but also actively from bone metastases, which occurred in the 18 PCa, although PCa bone metastases through lymphatic vessels were shown to be blocked by a cystic abscess formation or skin lipoma derived from lymphatic vessels. Among the 30 cas...

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Polymorphisms in PDCD1 gene are not associated with aplastic anemia in Chinese Han population

Rheumatology International — Sat, 17 Sep 2011 05:48:12 +0100

In conclusion, we show no association of selected SNPs in PDCD1 gene with AA in the Chinese Han population. Content Type Journal ArticleCategory Original ArticlePages 1-6DOI 10.1007/s00296-011-2127-0Authors Z. J. Ming, Department of Pharmacology, College of Pharmaceutical Science, Soochow University, Suzhou, People’s Republic of ChinaH. Hui, Department of Pharmacology, College of Pharmaceutical Science, Soochow University, Suzhou, People’s Republic of ChinaM. Miao, Department of Hematology, First Affiliated Hospital of Soochow University, Suzhou, People’s Republic of ChinaY. H. Qiu, Medical Biotechnology Institute, Soochow University, Suzhou, People’s Republic of ChinaX. G. Zhang, Medical Biotechnology Institute, Soochow University, Suzhou, People’s Republic of China ...

Gingival enlargement due to Cyclosporine A therapy in aplastic anaemia

Indian Journal of Pharmacology — Thu, 15 Sep 2011 04:00:00 +0100

Girish R Sabnis, Niteen D Karnik, Uma Sundar, Sikandar AdwaniIndian Journal of Pharmacology 2011 43(5):613-614 (Source: Indian Journal of Pharmacology)

Acceptable HLA-mismatching in unrelated donor bone marrow transplantation for patients with acquired severe aplastic anemia

Blood — Thu, 15 Sep 2011 04:00:00 +0100

We retrospectively analyzed the effect of HLA mismatching (HLA-A, -B, -C, -DRB1, -DQB1) with molecular typing on transplantation outcome for 301 patients with acquired severe aplastic anemia (SAA) who received an unrelated BM transplant through the Japan Marrow Donor Program. Additional effect of HLA-DPB1 mismatching was analyzed for 10 of 10 or 9 of 10 HLA allele-matched pairs (n = 169). Of the 301 recipient/donor pairs, 101 (33.6%) were completely matched at 10 of 10 alleles, 69 (23%) were mismatched at 1 allele, and 131 (43.5%) were mismatched at ≥ 2 alleles. Subjects were classified into 5 subgroups: complete match group (group I); single-allele mismatch group (groups II and III); multiple alleles restricted to HLA-C, -DRB1, and -DQB1 mismatch group (group IV); and others (group V)....

Fulminant invasive pulmonary mucormycosis with Rhizopus oryzae in a patient with severe aplastic anaemia and common variable immunodeficiency

Mycoses — Tue, 13 Sep 2011 04:00:00 +0100

(Source: Mycoses)

Parvovirus B19 infection associated with severe chronic aplastic anaemia—a case report

Journal of Hematopathology — Fri, 09 Sep 2011 05:49:08 +0100

We report a case of parvovirus B19-associated chronic aplastic anaemia in a 34-year-old female patient who presented with gum bleeding and ecchymotic patches over both legs and curdy vaginal discharge. She had pancytopenia on peripheral blood film examination and was subjected to bone marrow biopsy as the marrow aspirate showed peripheral blood only hence was not conclusive. Bone marrow biopsy was reported as AA, severe grade. The serological analysis performed later on revealed parvovirus B19 infection. Acute infection with B19V should be considered as a cause of acquired aplastic anaemia in individuals where other etiological factors are not elucidated. Content Type Journal ArticleCategory Case ReportPages 1-4DOI 10.1007/s12308-011-0113-9Authors Rajeev Sen, Department of Path...

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X‐linked VACTERL with hydrocephalus syndrome: Further delineation of the phenotype caused by FANCB mutations

American Journal of Medical Genetics Part A — Thu, 01 Sep 2011 04:00:00 +0100

AbstractX‐linked VACTERL‐hydrocephalus syndrome (X‐linked VACTERL‐H) is a rare disorder caused by mutations in the gene FANCB which underlies Fanconi Anemia (FA) complementation group B. Cells from affected males have increased chromosome breakage on exposure to DNA cross‐linking agents. Only five FANCB mutations found in six affected males, including an affected uncle and nephew, have been reported. We have identified FANCB mutations in a further four affected families. The VACTERL‐H phenotype segregates as an X‐linked recessive trait in three of these. Each mutation is predicted to truncate the FANCB open reading frame and results in highly skewed X‐inactivation in unaffected carrier females. Phenotypic data were available on six affected males. Comparison of the clinical...

Mismatched bone marrow transplantation for severe aplastic anaemia after liver transplantation for associated acute liver failure.

Annals of the Academy of Medicine, Singapore — Thu, 01 Sep 2011 04:00:00 +0100

Authors: Mali VP, Tan PL, Aw M, Loh LD, Quak SH, Madhavan K, Krishnan P PMID: 22065037 [PubMed - in process] (Source: Annals of the Academy of Medicine, Singapore)

Effect of stem cell source on outcomes after unrelated donor transplantation in severe aplastic anemia

Blood — Wed, 31 Aug 2011 23:00:00 +0100

Outcome after unrelated donor bone marrow (BM) transplantation for severe aplastic anemia (SAA) has improved, with survival rates now approximately 75%. Increasing use of peripheral blood stem and progenitor cells (PBPCs) instead of BM as a graft source prompted us to compare outcomes of PBPC and BM transplantation for SAA. We studied 296 patients receiving either BM (n = 225) or PBPC (n = 71) from unrelated donors matched at human leukocyte antigen-A, -B, -C, -DRB1. Hematopoietic recovery was similar after PBPC and BM transplantation. Grade 2 to 4 acute graft-versus-host disease risks were higher after transplantation of PBPC compared with BM (hazard ratio = 1.68, P = .02; 48% vs 31%). Chronic graft-versus-host disease risks were not significantly different after adjusting for age at tran...

Tcf-1 gene silence suppresses downstream gene expression in CD4+ T cells from bone marrow of aplastic anemia patients

Annals of Hematology — Wed, 31 Aug 2011 15:53:14 +0100

Abstract CD4+ T cells play a crucial role in the pathogenesis of acquired aplastic anemia (AA). Tcf-1 gene regulates T cell development and function, and it is significantly upregulated in the bone marrow CD4+ T cells from patients with acquired AA. To explore the role of Tcf-1 in the pathogenesis of AA, we knocked down Tcf-1 gene in CD4+ T cells of AA patients and studied the effects of Tcf-1 silencing on its downstream gene expression. Upon transfection of psiRNA into marrow CD4+ T cells from bone marrow of aplastic anemia patients, the expression of Tcf-1 was significantly knocked down; consequently, expressions of c-Myc and CD44 were also significantly reduced. Our results suggest that Tcf-1 may contribute to pathogenesis of AA by regulating downstream gene expression s...

Necrotizing Fasciitis After Acupuncture in a Patient with Aplastic Anemia

The Journal of Alternative and Complementary Medicine — Mon, 29 Aug 2011 18:55:13 +0100

The Journal of Alternative and Complementary Medicine Sep 2011, Vol. 17, No. 9: 871-874. (Source: The Journal of Alternative and Complementary Medicine)

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Infusion of autologous peripheral blood stem cells in an unrelated donor who developed severe aplastic anemia following stem cell donation

Bone Marrow Transplantation — Sun, 28 Aug 2011 23:00:00 +0100

Authors: H Frangoul, W H Navarro, D L Confer & M Jagasia (Source: Bone Marrow Transplantation)

Alemtuzumab with fludarabine and cyclophosphamide reduces chronic graft-versus-host disease after allogeneic stem cell transplantation for acquired aplastic anemia

Blood — Wed, 24 Aug 2011 23:00:00 +0100

We evaluated a novel alemtuzumab-based conditioning regimen in HSCT for acquired severe aplastic anemia (SAA). In a multicenter retrospective study, 50 patients received transplants from matched sibling donors (MSD; n = 21) and unrelated donors (UD; n = 29), using fludarabine 30 mg/m2 for 4 days, cyclophosphamide 300 mg/m2 for 4 days, and alemtuzumab median total dose of 60 mg (range:40-100 mg). Median age was 35 years (range 8-62). Overall survival at 2 years was 95% ± 5% for MSD and 83% for UD HSCT (p 0.34). Cumulative incidence of graft failure was 9.5% for MSD and 14.5% for UD HSCT. Full-donor chimerism (FDC) in unfractionated peripheral blood was 42%; no patient achieved CD3 FDC. Acute GVHD was observed in only 13.5% patients (all grade I-II) and only 2 patients (4%) developed ...

Equine ATG as the First-Line Treatment for Aplastic Anemia

Cancer Network — Wed, 24 Aug 2011 12:00:00 +0100

Equine ATG has been used for the treatment of severe aplastic anemia since the 1980s. Rabbit ATG is used in many parts of the world including South America, Japan, and European countries. The results of a randomized study of equine versus rabbit ATG showed that rabbit ATG was inferior to equine ATG. (Source: Cancer Network)

[Clinical analysis of 8 cases of hepatitis-associated aplastic anemia.]

Journal of Southern Medical University — Fri, 19 Aug 2011 23:00:00 +0100

CONCLUSIONS: HAAA may not have an obvious association with viral infections as by hepatitis virus A, B, C, D, or E, and its pathogenesis often involves abnormalities of the T cell immunity. Commonly with a poor prognosis, HAAA is associated with a high mortality rate. PMID: 21868343 [PubMed - as supplied by publisher] (Source: Journal of Southern Medical University)

Clopidogrel-Induced Neutropenia after Coronary Stenting: Is Cilostazol a Good Alternative?

Diagnostic and Therapeutic Endoscopy — Thu, 11 Aug 2011 14:15:38 +0100

We report a case of clopidogrel-induced bone marrow toxicity manifesting with severe neutropenia in a patient treated with multiple coronary stents and provide suggestions for an alternative treatment. (Source: Diagnostic and Therapeutic Endoscopy)

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Cefroxadine: First report of aplastic anaemia: case report

Reactions — Wed, 10 Aug 2011 17:36:32 +0100

(Source: Reactions)

Adult survivors of transplantation for aplastic anemia lived mostly healthy lives

HemOncToday.com — Wed, 10 Aug 2011 16:14:00 +0100

Sanders JE. Blood. 2011;118:1421-1428. (Source: HemOncToday.com)

Study on Clinical Characteristics and Follow-up Visit of Acquired Aplastic Anemia Associated with Parvovirus B19 Infection

Indian Journal of Pediatrics — Wed, 10 Aug 2011 15:41:42 +0100

Conclusions Parvovirus B19 infection contributes to the generation of AA, particularly in children aged 5–8 y. The AA induced may be mainly classified as serious and very serious type, with a course of disease less than 2 mo. Patients can be saved if B19-DNA is eliminated and the antibody is produced. Content Type Journal ArticlePages 1-6DOI 10.1007/s12098-011-0542-6Authors Jin-quan Wen, Children Disease Research Deparment, Xi’an Childern Hospital, Xi’an, Shanxi, People’s Republic of ChinaNan Zhou, Children Disease Research Deparment, Xi’an Childern Hospital, Xi’an, Shanxi, People’s Republic of ChinaDan Li, Children Disease Research Deparment, Xi’an Childern Hospital, Xi’an, Shanxi, People’s Republic of ChinaHai-ling Feng, Children Dise...

In Aplastic Anemia, Horse ATG Beats Rabbit (CME/CE)

MedPage Today Hematology/Oncology — Sat, 06 Aug 2011 16:43:06 +0100

(MedPage Today) -- Treatment with horse-derived anti-thymocyte globulin brought nearly double the hematologic response in acquired aplastic anemia as was seen with rabbit-derived ATG, data from a randomized trial showed. (Source: MedPage Today Hematology/Oncology)

Patients With Aplastic Anemia Benefit More From Standard Therapy Than From Newer Version

Health News from Medical News Today — Fri, 05 Aug 2011 07:00:00 +0100

A comparison clinical study of two aplastic anemia treatments found that ATGAM, currently the only licensed aplastic anemia drug in the United States, improved blood cell counts and survival significantly more than did Thymoglobulin, a similar but reportedly more potent treatment. The research was funded by the National Heart, Lung, and Blood Institute (NHLBI), a part of the National Institutes of Health; the study participants were treated and then followed at the NIH Clinical Center in Bethesda, Maryland. The study will appears in the August 4 New England Journal of Medicine... (Source: Health News from Medical News Today)

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Late effects among pediatric patients followed for nearly 4 decades after transplantation for severe aplastic anemia

Blood — Wed, 03 Aug 2011 23:00:00 +0100

This study evaluates late effects among 152 patients followed 1-38 years (median, 21.8 years). Transplantation-preparative regimes were mostly cyclophosphamide with or without antithymocyte globulin. Survival at 30 years for the acquired AA patients is 82%, and for the Fanconi anemia patients it is 58% (P = .01). Multivariate analysis demonstrated that chronic GVHD (P = .02) and Fanconi anemia (P = .03) negatively impacted survival. Two Fanconi patients and 18 acquired AA patients developed a malignancy that was fatal for 4. There was an increased incidence of thyroid function test abnormalities among those who received total body irradiation. Cyclophosphamide recipients demonstrated normal growth, basically normal development, and pregnancies with mostly normal offspring. Quality-of-life ...

Standard Aplastic Anemia Therapy Improves Patient Outcomes Better Than Newer Version

MedlinePlus Health News — Wed, 03 Aug 2011 22:12:55 +0100

Source: National Heart, Lung, and Blood Institute - Related MedlinePlus Page: Anemia (Source: MedlinePlus Health News)

Standard aplastic anemia therapy improves patient outcomes better than newer version, study finds

ScienceDaily Headlines — Wed, 03 Aug 2011 21:47:47 +0100

Horse versus Rabbit Antithymocyte Globulin in Acquired Aplastic Anemia

New England Journal of Medicine — Wed, 03 Aug 2011 21:00:15 +0100

New England Journal of Medicine, Volume 365, Issue 5, Page 430-438, August 2011. (Source: New England Journal of Medicine)

Traditional Chinese herbal medicine in the supportive management of patients with chronic cytopaenic marrow diseases - A phase I/II clinical study.

Complementary Therapies in Clinical Practice — Tue, 26 Jul 2011 02:43:36 +0100

This study was registered with Clinicaltrial.gov as NCT01224496. PMID: 21742281 [PubMed - in process] (Source: Complementary Therapies in Clinical Practice)

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Telomere biology in hematopoiesis and stem cell transplantation

Blood Reviews — Mon, 18 Jul 2011 04:00:00 +0100

Abstract: Telomeres are long (TTAGGG)n nucleotide repeats and an associated protein complex located at the end of the chromosomes. They shorten with every cell division and, thus are markers for cellular aging, senescence, and replicative capacity. Telomere dysfunction is linked to several bone marrow disorders, including dyskeratosis congenita, aplastic anemia, myelodysplastic syndrome, and hematopoietic malignancies. Hematopoietic stem cell transplantation (HSCT) provides an opportunity in which to study telomere dynamics in a high cell proliferative environment. Rapid telomere shortening of donor cells occurs in the recipient shortly after HSCT; the degree of telomere attrition does not appear to differ by graft source. As expected, telomeres are longer in recipients of grafts with long...

Non-Hodgkin lymphoma with hemorrhagic necrosis of the nasopharynx mimicking an abscess

American Journal of Otolaryngology — Mon, 18 Jul 2011 04:00:00 +0100

Abstract: Radiologic feature of primary nasopharyngeal lymphoma is a predominantly homogenous, nonnecrotic large tumor with no or minimal deep tissue invasion. To the best knowledge, nasopharyngeal lymphoma has not been presented with hemorrhagic necrosis. Thus, we report 2 cases of nasopharyngeal lymphoma with hemorrhagic necrosis mimicking an abscess. The patients had bleeding diathesis such as aplastic anemia or idiopathic portal hypertension. (Source: American Journal of Otolaryngology)

Zygomycosis in Two Hematologic Cases

Diagnostic and Therapeutic Endoscopy — Sun, 17 Jul 2011 14:51:40 +0100

Zygomycosis are invasive mould infections, rarely diagnosed in hematologic patients. Most of the cases published are in patients with prolonged neutropenia, along with other risk factors such as the use of prior broad-spectrum antibiotics (including new antifungal agents, such as voriconazole), diabetes mellitus (with or without ketoacidosis), malnutrition, iron overload (with or without the use of deferoxamine). These infections have poor prognosis due to the involvement of vital anatomic structures and late diagnosis. Until recent years, the treatment was based on high doses of amphotericin B plus surgical debridement. Here we present two patients with hematologic diseases (one with leukemia, the second with aplastic anemia) with an impaired immune system and the diagnosis of zygomycosis...

Favorable preliminary results using TLI/ATG‐based immunomodulatory conditioning for matched unrelated donor allogeneic hematopoietic stem cell transplantation in pediatric severe aplastic anemia

Pediatric Transplantation — Thu, 14 Jul 2011 23:00:00 +0100

Pillai A, Hartford C, Wang C, Pei D, Yang J, Srinivasan A, Triplett B, Dallas M, Leung W. Favorable preliminary results using TLI/ATG‐based immunomodulatory conditioning for matched unrelated donor allogeneic hematopoietic stem cell transplantation in pediatric severe aplastic anemia. Pediatr Transplantation 2011. © 2011 John Wiley & Sons A/S.Abstract: To assess whether a tolerance‐induction regimen could be applied for unrelated (MUD) HCT in SAA, we retrospectively reviewed our HCT experience using unmanipulated 10/10 HLA‐matched bone marrow grafts from MSD vs. MUD donors. Conditioning was CTX 200 mg/kg (CTX) + rabbit ATG 10 mg/kg (ATG) for MSD (n = 9) and TLI (800 cGy) + CTX/ATG for MUD HCT (n = 5). Immunoprophylaxis was CSA and short‐course MTX. M...

Susceptibility to aplastic anemia is associated with HLA-DRB1*1501 in an aboriginal population in Sabah, Malaysia.

Human Immunology — Thu, 30 Jun 2011 23:00:00 +0100

Authors: Dhaliwal JS, Wong L, Kamaluddin MA, Yin LY, Murad S The incidence of aplastic anemia is reported to be higher in Asia than elsewhere. We studied the frequency of human leukocyte antigen (HLA) DRB1 alleles in aplastic anemia patients from 2 genetically similar aboriginal groups, the Kadazan and the Dusun, and compared them with genetically matched community and hospital controls. HLA-DRB1*15 was significantly higher in the patients compared with controls (p = 0.005), confirming similar findings in Japanese and Caucasian studies. Further testing indicated a significantly higher frequency of HLA-DRB1*1501 in patients compared with controls (p = 0.0004) but no significant difference in the frequency of HLA-DRB1*1502. The high frequency of HLA-DRB1*15 in the Kadazan and Dusun popul...

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Do NK Cells Contribute to the Pathophysiology of Transplant‐Associated Thrombotic Microangiopathy?

American Journal of Transplantation — Wed, 29 Jun 2011 23:00:00 +0100

Transplant‐associated thrombotic microangiopathy (TA‐TMA) is a life‐threatening complication caused by the aggregation of platelets exposed to the thrombogenic subendothelial matrix of injured endothelial cells. Here, we present a case of a patient transplanted for idiopathic aplastic anemia with a T‐cell depleted hematopoietic stem cell graft from an HLA‐C mismatched unrelated donor. At day 7 posttransplant, she suffered from acute renal failure with hematuria. The presence of numerous schistocytes, an increased level of lactate dehydrogenase and a renal biopsy with multiple vascular injuries confirmed the diagnosis of severe TA‐TMA. At day 14, she developed graft versus host disease and died 7 months posttransplantation of multiorgan failure. At day 15, we observed a sizable ...

The Prognostic Value of p53 Mutation in Pediatric Marrow Hypoplasia

Diagnostic Pathology — Wed, 29 Jun 2011 23:00:00 +0100

Background: The tumor suppressor gene p53 is involved in the control of cell proliferation, particularly in stressed cells. p53 gene mutations are the most frequent genetic event found in human cancers. Fanconi Anemia (FA) is the most common representative of inherited bone marrow failure syndromes (IBMFS) with a leukemic propensity. p53 DNA alteration has not been studied before in Egyptian children with FA. Patients and methods: we investigated p53 DNA in the bone marrow and peripheral blood of forty children, FA (n=10), acquired aplastic anemia (AAA) (n=10), and immune thrombocytopenia ( ITP) as a control (n=20), using real-time PCR by TaqMan probe assay Results Mutation of p53 gene was demonstrated in the BM of 90% (9/10) of children with FA, compared to 10% (1/10) in AAA (p (Source: D...

SNP array-based karyotyping: differences and similarities between aplastic anemia and hypocellular myelodysplastic syndromes

Blood — Wed, 22 Jun 2011 23:00:00 +0100

In aplastic anemia (AA), contraction of the stem cell pool may result in oligoclonality, while in myelodysplastic syndromes (MDS) a single hematopoietic clone often characterized by chromosomal aberrations expands and outcompetes normal stem cells. We analyzed patients with AA (N = 93) and hypocellular MDS (hMDS, N = 24) using single nucleotide polymorphism arrays (SNP-A) complementing routine cytogenetics. We hypothesized that clinically important cryptic clonal aberrations may exist in some patients with BM failure. Combined metaphase and SNP-A karyotyping improved detection of chromosomal lesions: 19% and 54% of AA and hMDS cases harbored clonal abnormalities including copy-neutral loss of heterozygosity (UPD, 7%). Remarkably, lesions involving the HLA locus suggestive of clonal immune ...

Acquired severe aplastic anemia after H1N1 influenza virus vaccination successfully treated with allogeneic bone marrow transplantation

Annals of Hematology — Fri, 17 Jun 2011 10:55:39 +0100

Content Type Journal ArticlePages 1-2DOI 10.1007/s00277-011-1278-0Authors Irene Donnini, Haematology Unit and Bone Marrow Trasplantation Section, University of Florence and Careggi Hospital, Largo Brambilla 3, 50134 Florence, ItalyBarbara Scappini, Haematology Unit and Bone Marrow Trasplantation Section, University of Florence and Careggi Hospital, Largo Brambilla 3, 50134 Florence, ItalyStefano Guidi, Haematology Unit and Bone Marrow Trasplantation Section, University of Florence and Careggi Hospital, Largo Brambilla 3, 50134 Florence, ItalyGiovanni Longo, Haematology Unit and Bone Marrow Trasplantation Section, University of Florence and Careggi Hospital, Largo Brambilla 3, 50134 Florence, ItalyAlberto Bosi, Haematology Unit and Bone Marrow Trasplantation Section, University of Flore...

Lung Transplantation for Pulmonary Fibrosis in Dyskeratosis Congenita: Case Report and Systematic Literature Review

BMC Blood Disorders — Tue, 14 Jun 2011 23:00:00 +0100

Conclusion: Our current case and literature review indicate that pulmonary morbidity is one of the major contributors to poor quality of life and reduced long-term survival in DC. We suggest that lung transplantation be considered for patients with DC who develop pulmonary fibrosis with no concurrent evidence of multi-organ failure. (Source: BMC Blood Disorders)

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50 Years Ago in The Journal of Pediatrics: An Outbreak of Neonatal Deaths among Term Infants Associated with Administration of Chloramphenicol

The Journal of Pediatrics — Tue, 14 Jun 2011 15:13:03 +0100

Lischner HL, Seligman SJ, Krammer A, Parmelee AH. J Pediatr 1961;59:21. The antibiotic chloramphenicol was introduced in the early 1950s, and its broad spectrum and ability to reach effective serum and central nervous system concentrations when given intramuscularly and orally quickly led to its wide use. It was the subsequent recognition of two significant toxicities, however, that placed chloramphenicol on the back burner of pediatric therapeutics. The first was a severe, idiosyncratic aplastic anemia, occurring in approximately 1 of every 30 000 recipients. The second, the focus of the report by Lischner et al, was circulatory collapse when used in high doses in infants, which proved to be caused by slow glucuronidation and resulting toxic accumulation of drug in newborns. (Source: Th...

Telomere dysfunction and cell cycle checkpoints in hematopoietic stem cell aging.

International Journal of Hematology — Mon, 13 Jun 2011 23:00:00 +0100

Authors: Ju Z, Zhang J, Gao Y, Cheng T Stem cells are believed to be closely associated with tissue degeneration during aging. Studies of human genetic diseases and gene-targeted animal models have provided evidence that functional decline of telomeres and deregulation of cell cycle checkpoints contribute to the aging process of tissue stem cells. Telomere dysfunction can induce DNA damage response via key cell cycle checkpoints, leading to cellular senescence or apoptosis depending on the tissue type and developmental stage of a specific stem cell compartment. Telomerase mutation and telomere shortening have been observed in a variety of hematological disorders, such as dyskeratosis congenital, aplastic anemia, myelodysplastic syndromes and leukemia, in which the hematopoietic stem ce...

Diagnosis and outcome of neutropenic enterocolitis: experience in a single tertiary pediatric surgical center in China

Pediatric Surgery International — Sat, 11 Jun 2011 06:30:35 +0100

Conclusion NE is a life-threatening gastrointestinal complication of neutropenia. Physicians might remain vigilant and consider NE in any neutropenic patient rather than only in oncologic patients. It has typical clinical presentation and CT can provide clear delineation for diagnosis. Early recognition and progressed management have reduced mortality. Most children with NE may be treated without surgery with favorable outcome. Content Type Journal ArticlePages 1-5DOI 10.1007/s00383-011-2938-9Authors Kai Li, Children’s Hospital of Fudan University, 399 Wang Yuan Road, Shanghai, 201102 People’s Republic of ChinaShan Zheng, Children’s Hospital of Fudan University, 399 Wang Yuan Road, Shanghai, 201102 People’s Republic of ChinaKuiran Dong, Children’s Hospita...

Clinical observations on intensive immunosuppressive therapy combined with umbilical cord blood support for the treatment of severe aplastic anemia

Journal of Hematology and Oncology — Thu, 09 Jun 2011 23:00:00 +0100

Conclusion: Enhanced, intensive, immuno-suppressive therapy with umbilical cord blood support may be an effective option for SAA therapy. (Source: Journal of Hematology and Oncology)

Musculoskeletal Manifestations of Chronic Anemias

Seminars in Musculoskeletal Radiology — Fri, 03 Jun 2011 04:00:00 +0100

This article provides an overview of the current use of diagnostic imaging modalities in the evaluation of a heterogeneous group of disorders causing chronic anemias by impaired blood cell production (inherited bone marrow failure syndromes of childhood, aplastic anemia and myelodysplastic syndromes, β-thalassemia) or increased blood cell destruction (sickle cell disease). During the course of these disorders, various musculoskeletal abnormalities can be encountered, including marrow hyperplasia, reversion of yellow marrow to red marrow, growth disturbances, and, occasionally, extramedullary hematopoiesis. Diagnostic imaging may help the clinician to identify specific complications related to either the disease (e.g., bone infarction and acute osteomyelitis in sickle cell disease) or tran...

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Effect of busui shengxue granule (see text) on chronic aplastic anemia patients' hematopoietic adhesion molecule VLA-6/CD49f and its ligand laminin.

Journal of Traditional Chinese Medicine — Wed, 01 Jun 2011 04:00:00 +0100

CONCLUSION: The VLA-6/CD49f and Ln expressions of CAA patients are abnormal. The treatment with Busui Shengxue Granule makes both of them improved. PMID: 21977812 [PubMed - in process] (Source: Journal of Traditional Chinese Medicine)

Costs and consequences of immunosuppressive therapy in children with aplastic anemia.

Haematologica — Tue, 31 May 2011 23:00:00 +0100

Authors: Shereck EB, Deyell RJ, Kurre P PMID: 21632841 [PubMed - in process] (Source: Haematologica)

Clinical course of patients with aplastic anemia or myelodysplastic syndrome associated with persistent neutropenia

Hematological Oncology — Tue, 31 May 2011 23:00:00 +0100

In conclusion, severe neutropenia was significantly associated with IE in patients with AA or MDS, and IE might be lethal. When we only considered patients with MDS, the neutrophil count alone could not be used to predict the prognosis. Copyright © 2011 John Wiley & Sons, Ltd. (Source: Hematological Oncology)

Severe aplastic anemia following liver transplantation in a patient with non-A-E fulminant hepatitis.

The Turkish Journal of Gastroenterology — Tue, 31 May 2011 23:00:00 +0100

Authors: Tümgör G, Aydoğdu S, Karapinar DY, Karapinar B, Arikan C, Kiliç M, Aydinok Y Aplastic anemia is a rare complication after liver transplantation. Its incidence has been estimated to be 0.007%. Aplastic anemia was observed in 23.2 33% of patients who underwent liver transplantation for fulminant non-A-E viral hepatitis. In this paper, we describe a child suffering from aplastic anemia who eventually died from sepsis after liver transplantation for non-A-E fulminant hepatic failure. PMID: 21805427 [PubMed - in process] (Source: The Turkish Journal of Gastroenterology)

Nutritional support in the treatment of aplastic anemia

Nutrition — Mon, 30 May 2011 04:00:00 +0100

Conclusion: Specific nutritional supplements accelerated rehabilitation of AA mice and can be used as nutritional support in the treatment of AA. (Source: Nutrition)

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Long-Term Survival and Late Deaths After Allogeneic Hematopoietic Cell Transplantation [Hematologic Malignancies]

Journal of Clinical Oncology — Thu, 26 May 2011 23:00:00 +0100

Conclusion The prospect for long-term survival is excellent for 2-year survivors of allogeneic HCT. However, life expectancy remains lower than expected. Performance of HCT earlier in the course of disease, control of GVHD, enhancement of immune reconstitution, less toxic regimens, and prevention and early treatment of late complications are needed. (Source: Journal of Clinical Oncology)

The Third Consensus Conference on the treatment of aplastic anemia.

International Journal of Hematology — Thu, 26 May 2011 23:00:00 +0100

Authors: Kojima S, Nakao S, Young N, Bacigalupo A, Gerard G, Hirano N, Maciejewski J, Deeg J, Marsh J, Zhang FK, Lee JW, Ozawa K PMID: 21617886 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)

Native valve Aspergillus endocarditis in two patients with aplastic anaemia

The Aspergillus Website - articles — Thu, 26 May 2011 14:19:39 +0100

Petrikkos GL, Skiada A, Samonis G, Mavroudis D, Daikos GL (Source: The Aspergillus Website - articles)

Syndrome complex of bone marrow failure and pulmonary fibrosis predicts germline defects in telomerase

Blood — Wed, 25 May 2011 23:00:00 +0100

We examined whether the cooccurrence of bone marrow failure and pulmonary fibrosis in the same individual or family enriches for the presence of a telomerase mutation. Ten consecutive individuals with a total of 36 family members who fulfilled these criteria carried a germline mutant telomerase gene (100%). The mean age of onset for individuals with AA was significantly younger than that for those with pulmonary fibrosis (14 vs 51; P < .0001). Families displayed autosomal dominant inheritance and there was an evolving pattern of genetic anticipation, with the older generation primarily affected by pulmonary fibrosis and successive generations by bone marrow failure. The cooccurrence of AA and pulmonary fibrosis in a single patient or family is highly predictive for the presence of a ger...

Hematologic adverse events associated with temozolomide

Cancer Chemotherapy and Pharmacology — Wed, 25 May 2011 16:01:56 +0100

Conclusion The hematologic toxicity profile of TMZ differs from that of other alkylating agents. TMZ HAEs are emerging as significant concerns. Among alkylating agents, AA appears unique to TMZ, and the high rate warrants disclosure of patients. The duration of TMZ exposure prior to the development of AA may be quite short. The risk of AML/MDS is low, but the length of follow-up is insufficient to assess the true risk. Content Type Journal ArticlePages 1-7DOI 10.1007/s00280-011-1679-8Authors J. Lee Villano, Department of Medicine, Section of Hematology/Oncology, University of Illinois at Chicago, 909 S. Wolcott Ave. Rm. 3133 (M/C 734), Chicago, IL 60612, USANathalie Letarte, Faculty of Pharmacy, Universite de Montreal, Montreal, CanadaJanny M. Yu, Department of Pha...

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Haemolysis after treatment with intravenous immunoglobulin due to anti‐A

Transfusion Medicine — Tue, 24 May 2011 17:36:29 +0100

Conclusion: These cases illustrate the potential severity of haemolysis after IVIG because of passively transferred antibodies to blood group antigens. Lack of recognition of IVIG as a cause for haemolysis by clinicians may be further confounded if routine testing fails to detect the passively transferred ABO blood group antibodies. (Source: Transfusion Medicine)

Paroxysmal nocturnal hemoglobinuria and pregnancy before the eculizumab era: the French experience.

Haematologica — Sun, 22 May 2011 23:00:00 +0100

Conclusions. Pregnancy during paroxysmal nocturnal hemoglobinuria is associated with increased maternal and fetal mortality rates (8% and 4%, respectively, in this series). Maternal mortality is related to postpartum thromboses. Prophylactic anticoagulation is recommended during pregnancy and for 6 weeks postpartum. PMID: 21606169 [PubMed - as supplied by publisher] (Source: Haematologica)

Efficacy of rabbit antithymocyte globulin in severe aplastic anemia.

Haematologica — Sun, 22 May 2011 23:00:00 +0100

Conclusions. Despite reports suggesting differences in biologic activity of different anti-thymocyte globulin preparations, rabbit and horse anti-thymocyte globulin appear to have a similar efficacy for upfront treatment of severe aplastic anemia.The registration number for this trial at clincaltrial.gov is NCT01231841. PMID: 21606164 [PubMed - as supplied by publisher] (Source: Haematologica)

[Expression of CD133 in the bone marrow of patients with myelodysplastic syndrome and its clinical significance.]

Journal of Southern Medical University — Thu, 19 May 2011 23:00:00 +0100

CONCLUSIONS: Advanced MDS patients are characterized by an increase of CD133-expressing cells, suggesting the value of CD133 in the diagnosis of RAEB. CD34(+)/CD38- cells do not show a significant value in the diagnosis of MDS. PMID: 21602141 [PubMed - as supplied by publisher] (Source: Journal of Southern Medical University)

Paroxysmal Nocturnal Hemoglobinuria after Autologous Stem Cell Transplantation: Extinction of the Clone during Treatment with Eculizumab - Pathophysiological Implications of a Unique Clinical Case.

Acta Haematologica — Wed, 18 May 2011 23:00:00 +0100

We report a previously undescribed case of PNH occurring after autologous stem cell transplantation (ASCT) in a patient affected by relapsing non-Hodgkin's lymphoma. The intensive chemotherapy and the ASCT resulted in a contraction of the effective hematopoietic stem cell (HSC) pool and a derangement of the immune system. The delayed engraftment and the BM hypoplasia represented a favorable environment for the expansion of the pathological clone. This case is paradigmatic even for the unexpected trend of the PNH clone during treatment with the terminal complement inhibitor eculizumab; in fact, the clone reduced until undergoing unexpected extinction, i.e. the recovery of normal hematopoiesis. Eculizumab seems not to play a direct role in HSC kinetics; the clinical remission probably occurr...

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Inherited bone marrow failure syndromes

Journal of Hematopathology — Thu, 12 May 2011 16:45:38 +0100

Abstract The inherited bone marrow failure syndromes are a diverse group of disorders characterized by BM failure usually in association with one or more somatic/physical abnormality. Over the last two decades, the genes responsible for many of these have been identified. The significant advances in their molecular basis have provided insights into several biological pathways, such as DNA repair and telomere maintenance, of importance in human physiology. They have also provided a link between the inherited and idiopathic forms of bone marrow failure syndromes; for example, some patients with idiopathic forms of aplastic anemia have mutations in genes first identified in the classic forms of dyskeratosis congenita. Clinical similarities (bone marrow failure, developmental ...

CD20-negative Epstein-Barr virus-associated post-transplant lymphoproliferative disease refractory to rituximab in a patient with severe aplastic anemia.

International Journal of Hematology — Tue, 10 May 2011 23:00:00 +0100

Authors: Muramatsu H, Takahashi Y, Shimoyama Y, Doisaki S, Nishio N, Ito Y, Hama A, Shimada A, Yagasaki H, Ito M, Kojima S Epstein-Barr virus-associated post-transplant lymphoproliferative disease (EBV-PTLD) is a life-threatening complication following allogeneic hematopoietic stem cell transplantation (HSCT). Monitoring of EBV DNA in high-risk patients with subsequent pre-emptive rituximab treatment is highly effective, and can prevent EBV-associated disease following HSCT. Here, we report a 10-year-old girl with aplastic anemia who developed CD20 negative EBV-PTLD after unrelated bone marrow transplantation that was refractory to rituximab treatment. Similar to other types of lymphoma, the absence of CD20 antigen is likely to be characteristic of rituximab-refractory EBV-PTLD. PM...

Novel Immunosuppressive Strategies for Bone Marrow Failure Syndromes: A Focus on Alemtuzumab.

Mini Reviews in Medicinal Chemistry — Tue, 10 May 2011 23:00:00 +0100

Authors: Selleri C, Serio B, Risitano AM Acquired bone marrow failure syndromes (BMFS) are a heterogeneous group of hematological disorders characterized by impaired bone marrow function and subsequent cytopenia of one or more blood cell lineages [1,2]. The well-accepted pathogenic mechanism of the typical bone marrow failure - aplastic anemia (AA)- is a T cell mediated immune attack targeting the hematopoietic tissue [3]. This pathogenic mechanism is at least partially shared by other bone marrow failure syndromes, such as lineage-restricted aplasias and some myelodysplastic syndromes. Thus, for these disorders immunosuppression (IS) is the pivotal etiologic treatment. While the standard IS regimen include the heterologous anti-thymocyte globulin [4], here we review the recent data on...

Impact of Immunogenetic Polymorphisms in Bone Marrow Failure Syndromes.

Mini Reviews in Medicinal Chemistry — Tue, 10 May 2011 23:00:00 +0100

Conclusion: Our findings suggest that alterations in KIR/KIR-L matching, such as increased 3DL2 and decreased 2DS1 mismatch, and in the polymorphisms of TGFβ1, IFN-γ, TNF- α and IL-10 may account for the propensity to immunemediated killing of hematopoietic stem cells and/or ineffective hematopoiesis characteristic of AA and MDS. Further studies are needed to elucidate whether these immunogenetic traits may be involved in increased risk of developing immune-mediated BMF. PMID: 21561401 [PubMed - as supplied by publisher] (Source: Mini Reviews in Medicinal Chemistry)

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Congenital pancytopenia and absence of B lymphocytes in a neonate with a mutation in the ikaros gene

Pediatric Blood and Cancer — Wed, 04 May 2011 23:00:00 +0100

ConclusionsOur report describes a novel case of congenital pancytopenia associated with mutation of the IKAROS gene. Furthermore, these data suggest a critical role of IKAROS in human hematopoiesis and immune development. Pediatr Blood Cancer © 2011 Wiley‐Liss, Inc. (Source: Pediatric Blood and Cancer)

Camp gives sick kids new world view

CNN.com - Health — Mon, 02 May 2011 15:50:56 +0100

When Catherine MacLean was 4 years old, learned she had aplastic anemia. Hole in the Wall Gang Camps, started by Paul Newman, helped her transition to more normal life. (Source: CNN.com - Health)

Camp: New outlook for sick kids

CNN.com - Health — Mon, 02 May 2011 15:06:43 +0100

When Catherine MacLean was 4 years old, learned she had aplastic anemia. Hole in the Wall Gang Camps, started by Paul Newman, helped her transition to more normal life. (Source: CNN.com - Health)

Summer camp for sick kids

CNN.com - Health — Mon, 02 May 2011 12:47:42 +0100

When Catherine MacLean was 4 years old, learned she had aplastic anemia. Hole in the Wall Gang Camps, started by Paul Newman, helped her transition to more normal life. (Source: CNN.com - Health)

[Fanconi anemia: cytogenetic diagnosis of 40 cases].

Acta Medica Portuguesa — Sun, 01 May 2011 04:00:00 +0100

Authors: Porto B, Sousa R, Ponte F, Torgal A, Campilho F, Campos A, Gonçalves C, Barbot J Abstract Fanconi Anemia (FA) is a rare recessive disorder clinically characterized by progressive bone marrow failure, diverse congenital malformations and increased predisposition to cancer. Given the late onset of anemia, relatively to other cytopenias, and the high variability in the phenotype, a correct clinical diagnosis is difficult, and may be delayed or even missed. This fact may be prejudicial to patients, due to the need of avoiding exposure to toxic agents, programming the transplantation of hematopoietic progenitor cells and screening of neoplasia associated with the disease. Given the high genetic variability (thirteen complementation groups have been identified, each with genes ...

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Aplastic anemia and clopidogrel/ Nonfatal aplastic anemia associated with clopidogrel.

Anadolu Kardiyol Der... — Sat, 30 Apr 2011 23:00:00 +0100

Authors: Civriz Bozdağ S, Ozcan M PMID: 21501990 [PubMed - in process] (Source: Anadolu Kardiyol Der...)

A randomized controlled study in patients with newly diagnosed severe aplastic anemia receiving antithymocyte globulin (ATG), cyclosporine, with or without G-CSF: a study of the SAA Working Party of the European Group for Blood and Marrow Transplantation

Blood — Wed, 27 Apr 2011 23:00:00 +0100

This study was registered at www.clinicaltrials.gov as NCT01163942. (Source: Blood)

The replication rate of human hematopoietic stem cells in vivo

Blood — Wed, 27 Apr 2011 23:00:00 +0100

In this report, we analyze the changing ratio with age of maternal/paternal X-chromosome phenotypes in blood cells from females and infer that human HSCs replicate on average once every 40 weeks (range, 25-50 weeks). We then confirm this estimate with 2 independent approaches, use the estimate to simulate human hematopoiesis, and show that the simulations accurately reproduce marrow transplantation data. Our simulations also provide evidence that the number of human HSCs increases from birth until adolescence and then plateaus, and that the ratio of contributing to quiescent HSCs in humans significantly differs from mouse. In addition, they suggest that human marrow failure, such as the marrow failure that occurs after umbilical cord blood transplantation and with aplastic anemia, results ...

Management of Aplastic Anemia in a Woman During Pregnancy

Medscape Today Headlines — Mon, 25 Apr 2011 11:05:36 +0100

Aplastic anemia is a potentially life-threatening condition which may manifest during pregnancy. While the prognosis is usually good, serious complications arose in this patient. Journal of Medical Case Reports (Source: Medscape Today Headlines)

Interleukin‐27 enhances the production of tumour necrosis factor‐α and interferon‐γ by bone marrow T lymphocytes in aplastic anaemia

British Journal of Haematology — Wed, 20 Apr 2011 23:00:00 +0100

In conclusion, our data suggest that elevated IL‐27 and IL‐27‐induced TNF‐α and IFN‐γ overproduction might be involved in the pathogenesis of AA. (Source: British Journal of Haematology)

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Leflunomide: Aplastic anaemia: case report

Reactions — Sat, 16 Apr 2011 17:06:15 +0100

(Source: Reactions)

Myelodysplasia and leukemia of Fanconi anemia are associated with a specific pattern of genomic abnormalities that includes cryptic RUNX1/AML1 lesions

Blood — Wed, 13 Apr 2011 23:00:00 +0100

Fanconi anemia (FA) is a genetic condition associated with bone marrow (BM) failure, myelodysplasia (MDS), and acute myeloid leukemia (AML). We studied 57 FA patients with hypoplastic or aplastic anemia (n = 20), MDS (n = 18), AML (n = 11), or no BM abnormality (n = 8). BM samples were analyzed by karyotype, high-density DNA arrays with respect to paired fibroblasts, and by selected oncogene sequencing. A specific pattern of chromosomal abnormalities was found in MDS/AML, which included 1q+ (44.8%), 3q+ (41.4%), –7/7q (17.2%), and 11q– (13.8%). Moreover, cryptic RUNX1/AML1 lesions (translocations, deletions, or mutations) were observed for the first time in FA (20.7%). Rare mutations of NRAS, FLT3-ITD, MLL-PTD, ERG amplification, and ZFP36L2-PRDM16 translocation, but no TP53, T...

Successful Treatment of Methimazole-Induced Severe Aplastic Anemia by Granulocyte Colony-Stimulating Factor, Methylprednisolone, and Cyclosporin

Computational Intelligence and Neuroscience — Mon, 11 Apr 2011 13:54:13 +0100

A 52-year-old Japanese woman was examined because of general malaise, weight loss and a lump in her left breast. She was diagnosed with cancer of the left breast and Graves' disease, and was administered methimazole (MMI). A left mastectomy was performed for the breast cancer. She presented with a high fever and peripheral blood examination revealed a severe pancytopenia. She was diagnosed with severe aplastic anemia, and administered G-CSF, however, the treatment was unsuccessful. Thus, oral methyprednisolone and cyclosporin were added. There was a remarkable improvement in the peripheral blood count. (Source: Computational Intelligence and Neuroscience)

A founder mutation in the MPL gene causes congenital amegakaryocytic thrombocytopenia (CAMT) in the Ashkenazi Jewish population.

Blood Cells, Molecules and Diseases — Sun, 10 Apr 2011 23:00:00 +0100

We report here the identification and characterization of a founder mutation in MPL in the Ashkenazi Jewish (AJ) population. This mutation, termed c.79+2T>A, is a T to A transversion in the invariant second base of the intron 1 donor splice site. Analysis of a random sample of 2018 individuals of AJ descent revealed a carrier frequency of approximately 1 in 75. Genotyping of six loci adjacent to the MPL gene in the proband and in the 27 individuals identified as carriers of the c.79+2T>A mutation revealed that the presence of this mutation in the AJ population is due to a single founder. The observed carrier frequency predicts an incidence of CAMT in the AJ population of approximately 1 in 22,500 pregnancies. The identification of this mutation will enable population carrier testing ...

Lentiviral gene transfer regenerates hematopoietic stem cells in a mouse model for Mpl-deficient aplastic anemia

Blood — Wed, 06 Apr 2011 23:00:00 +0100

In this study, we used the Mpl knockout (Mpl–/–) mouse model and expressed Mpl from newly developed lentiviral vectors specifically in the physiologic Mpl target populations, namely, HSCs and megakaryocytes. After validating lineage-specific expression in vivo using lentiviral eGFP reporter vectors, we performed bone marrow transplantation of transduced Mpl–/– bone marrow cells into Mpl–/– mice. We show that restoration of Mpl expression from transcriptionally targeted vectors prevents lethal adverse reactions of ectopic Mpl expression, replenishes the HSC pool, restores stem cell properties, and corrects platelet production. In some mice, megakaryocyte counts were atypically high, accompanied by bone neo-formation and marrow fibrosis. Gene-corrected Mpl...

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Rhizomucor and Scedosporium Infection Post Hematopoietic Stem-Cell Transplant

Journal of Oncology — Tue, 05 Apr 2011 14:44:42 +0100

We report a case of a 17-year-old male patient diagnosed with severe idiopathic acquired aplastic anemia who developed fungal pneumonitis due to Rhizomucor sp. and rhinoencephalitis due to Scedosporium apiospermum 6 and 8 months after undergoing allogeneic hematopoietic stem-cell transplant from an HLA-matched unrelated donor. Discussion highlights risk factors for invasive fungal infections (i.e., mucormycosis and scedosporiosis), its clinical features, and the factors that must be taken into account to successfully treat them (early diagnosis, correction of predisposing factors, aggressive surgical debridement, and antifungal and adjunctive therapies). (Source: Journal of Oncology)

ATG plus cyclosporine reduces all-cause mortality in patients with severe aplastic anemia: systematic review and meta-analysis

NeLM - Disease Focused Reviews — Mon, 04 Apr 2011 23:00:00 +0100

The objectives and inclusion criteria of the review were clear and relevant sources were searched for studies. A specific attempt was made to retrieve unpublished studies, but no test for publication bias appeared to be conducted. It was unclear whether the search was restricted by language. Steps were taken to minimise the risk of reviewer bias and error by having more than one reviewer involved in selection of studies, assessment of validity and data extraction. Some relevant criteria were used to assess study validity, but no ... (Source: NeLM - Disease Focused Reviews)

Transfusion Medicine and the Pregnant Patient

Hematology/Oncology Clinics of North America — Tue, 29 Mar 2011 14:23:56 +0100

Alloimmunity in pregnancy is the basis for two of the major complications of pregnancy in transfusion medicine: hemolytic disease of the fetus and newborn (HDFN), and fetal and neonatal alloimmune thrombocytopenia (FNAIT). Use of Rh(D) immune globulin has dramatically reduced the incidence of HDFN in Rh(D)-mismatched pregnancies. Treatment of HDFN may involve intrauterine transfusion, with fetal and neonatal survival rates of 70% to 90%. Treatments for FNAIT include immune globulin, steroids, or in severe cases, intrauterine platelet transfusions. Transfusion medicine is central to the management of pregnancy-associated complications such as postpartum hemorrhage, parvovirus B19 infection, hemoglobinopathies, and aplastic anemia. (Source: Hematology/Oncology Clinics of North America)

Anemia in Pregnancy

Hematology/Oncology Clinics of North America — Tue, 29 Mar 2011 14:23:55 +0100

Anemia in pregnancy is a global health problem affecting nearly half of all pregnant women worldwide. High fetal demands for iron render iron deficiency the most common cause of anemia of pregnancy, with other micronutrient deficiencies contributing less frequently. In certain geographical populations, human pathogens such as hookworm, malarial parasite and human immunodeficiency virus are important factors in anemia of pregnancy. The hemoglobinopathies, sickle cell disease and thalassemia, represent diverse causes of anemia of pregnancy, requiring specialized care. Aplastic anemia is a rare, morbid cause of anemia of pregnancy and is managed with transfusions until the completion of pregnancy. (Source: Hematology/Oncology Clinics of North America)

Natural history of paroxysmal nocturnal hemoglobinuria clones in patients presenting as aplastic anemia

European Journal of Haematology — Mon, 28 Mar 2011 23:00:00 +0100

Conclusion: The risk of developing clinically significant PNH after HiCy therapy appears to be low in AA patients with PNH clones, especially for those with small initial PNH clones and for those who respond to HiCy therapy. (Source: European Journal of Haematology)

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An anticlastogenic function for the Polycomb Group gene Bmi1 [Cell Biology]

Proceedings of the National Academy of Sciences — Mon, 28 Mar 2011 23:00:00 +0100

BMI1 is a key component of multiprotein Polycomb repression complex 1 (PRC1), and its disruption in mice induces severe aplastic anemia by early adulthood. The contributing mechanisms responsible for this phenotype remain elusive. Here we show that transformed human cell lines as well as primitive hematopoietic cells exhibit a high frequency of spontaneous chromosome breaks upon BMI1 depletion and are hypersensitive to genotoxic agents. Consistent with these observations, we found that BMI1 is recruited rapidly to DNA damage foci where it blocks transcriptional elongation. We also show that BMI1 contributes to homologous recombination DNA repair and is required for checkpoint recovery. Taken together, our results suggest that BMI1 is critical for the maintenance of chromosome integrity in ...

The Patterns of MHC Association in Aplastic and Non-aplastic Paroxysmal Nocturnal Hemoglobinuria

Archivum Immunologiae et Therapiae Experimentalis — Sun, 27 Mar 2011 05:55:05 +0100

Abstract The deficiency of glycosyl-phosphatidylinositol (GPI)-anchored proteins in plasma membranes of PIG-A gene mutated hematopoietic stem cells (HSCs) is so far insufficient to explain the domination of paroxysmal nocturnal hemoglobinuria (PNH) clone over the normal HSC. We attempted to elucidate possible link between MHC and initial severe aplastic anemia (ISAA/PNH) type and non-aplastic (n/PNH) outcome of PNH. In 50 PNH patients assigned as ISAA/PNH (n = 13), n/PNH (n = 33) or nonassigned (n = 4) and 200 ethnically matched controls we analyzed MHC associations. Our data confirmed strong associations of DRB1*15:01 (RR = 3.51, p = 0.0011) and DQB1*06:02 (RR = 7.09, p = 0.000026) alleles, especially with ...

Acute Liver Failure and Aplastic Anemia in an 11-Year-Old Girl

Digestive Diseases and Sciences — Fri, 25 Mar 2011 18:55:30 +0100

Content Type Journal ArticlePages 1-4DOI 10.1007/s10620-011-1678-yAuthors Ann Ming Yeh, Division of Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Stanford University Medical Center, Lucile Packard Children’s Hospital at Stanford, 750 Welch Road, Suite 116, Palo Alto, CA 94304-0126, USAAmirkaveh Mojtahed, Department of Pathology, Stanford University Medical Center, 300 Pasteur Drive, H2110, Stanford, CA 94305, USADorsey Bass, Division of Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Stanford University Medical Center, Lucile Packard Children’s Hospital at Stanford, 750 Welch Road, Suite 116, Palo Alto, CA 94304-0126, USA Journal Digestive Diseases and SciencesOnline ISSN 1573-2568Print ISSN 0163-2116 (Source: Digestive Disease...

Research paper: Mortality of US pentachlorophenol production workers through 2005.

Chemosphere — Thu, 24 Mar 2011 00:00:00 +0100

Authors: Ruder AM, Yiin JH A cohort of 2122 US pentachlorophenol (PCP) production workers from four plants in the National Institute for Occupational Safety and Health Dioxin Registry was exposed to PCP and to polychlorinated dibenzo-p-dioxin and dibenzofuran contaminants of PCP production. A subcohort of 720 was also exposed to 2,3,7,8-tetrachlorodibenzodioxin, a contaminant of trichlorophenol (TCP) while using TCP or a TCP derivative. PCP and several production contaminants have been implicated as animal carcinogens. A priori hypotheses were that the cohort would have elevated standardized mortality ratios (SMRs) for aplastic anemia, soft-tissue sarcoma, and non-Hodgkin lymphoma, as suggested by human studies, and for leukemia and liver, adrenal, thyroid, and parathyroid cancer, as s...

Successful immunosuppressive and iron chelation therapy for a severe aplastic anemia patient undergoing hemodialysis due to chronic renal failure.

International Journal of Hematology — Wed, 23 Mar 2011 00:00:00 +0100

Authors: Hiraga J, Sakemura R, Yamashita H, Suzuki T, Kitagawa S, Takakuwa Y, Mizuno S PMID: 21431347 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)

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Aplastic anemia successfully treated with rituximab: The possible role of aplastic anemia‐associated autoantibodies as a marker for response

European Journal of Haematology — Tue, 22 Mar 2011 17:50:23 +0100

AbstractA 1‐year‐old Japanese male developed hepatitis‐associated aplastic anemia (AA) and anti‐thymocyte globulin (ATG) plus cyclosporine A (CsA) was administered without any appreciable effects. Laboratory examination of the patient’s serum obtained before therapy revealed various autoantibodies, such as PA‐IgG, anti‐platelets, anti‐single‐stranded DNA (ssDNA), and anti‐double‐stranded DNA (dsDNA) antibodies (Abs) in addition to anti‐DRS‐1 Abs and anti‐moesin Abs, both of which are known to be detectable in approximately 40% of all patients presenting with AA. He was therefore treated with 17.5 mg/kg/d rituximab 5.5 months after ATG/CsA therapy. The same rituximab therapy was repeated 3 times once a month thereafter. His neutrophil counts started to increase 5...

Relapse of aplastic anemia in children after immunosuppressive therapy: a report from the Japan Childhood Aplastic Anemia Study Group.

Haematologica — Mon, 21 Mar 2011 00:00:00 +0100

Conclusions. In the present study, the cumulative incidence of relapse at ten years was relatively low compared with other studies that mainly consisted of adult patients. To establish optimal therapy for these patients, a multicenter prospective study is warranted. PMID: 21422115 [PubMed - as supplied by publisher] (Source: Haematologica)

Ophthalmic chloramphenicol - is it safe in breastfeeding?

NeLM - Medicines Q and A — Fri, 18 Mar 2011 00:00:00 +0100

Source: Trent Medicines Information Service Area: Evidence > Medicines Q & A Ophthalmic chloramphenicol products,eye drops and eye ointment, are routinely used, and considered to be the treatment of choice for superficial eye infections. There is conflicting evidence that ophthalmic chloramphenicol can precipitate blood dyscrasias, especially aplastic anaemia, due to systemic absorption, as this reaction is not dose-related. However, the balance of evidence suggests that the association of ophthalmic chloramphenicol and aplastic anaemia has not been established. This conclusion, therefore, also applies to the use of ophthalmic chloramphenicol in mothers who are breastfeeding their infants. It does, however, remain a theoretical risk. Systemic chl...

Successful sustained engraftment after reduced-intensity umbilical cord blood transplantation for adult patients with severe aplastic anemia

Blood — Thu, 17 Mar 2011 00:00:00 +0100

We retrospectively analyzed 12 consecutive adult severe aplastic anemia patients who received unrelated umbilical cord blood transplantation after a reduced-intensity conditioning regimen (RI-UCBT). The conditioning regimen consisted of 125 mg/m2 fludarabine, 80 mg/m2 melphalan, and 4 Gy of total body irradiation. The median infused total nucleated cell number and CD34+ cell number were 2.50 x 107/kg and 0.76 x 105/kg, respectively. Eleven of the 12 patients achieved primary neutrophil and platelet engraftment. All patients who achieved engraftment had complete hematologic recovery with complete donor chimerism, except for one patient who developed late graft failure 3 years after RI-UCBT. Two of the 12 patients died of idiopathic pneumonia syndrome, and the remaining 10 patients are alive...

Erratum to: A case of Klinefelter syndrome with aplastic anemia.

International Journal of Hematology — Fri, 04 Mar 2011 00:00:00 +0100

Authors: Xu C, Zhang CY, Chen N, Sun X, Xiao Y, Gao L, Zhao JJ PMID: 21374074 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)

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Hematopoietic cell transplantation with autologous cord blood in patients with severe aplastic anemia: An opportunity to revisit the controversy regarding cord blood banking for private use

Pediatric Blood and Cancer — Wed, 02 Mar 2011 00:00:00 +0100

AbstractThe controversy surrounding private banking of umbilical cord blood units (CBU), as a safeguard against future malignancy or other life‐threatening conditions, raises many questions in pediatric clinical practice. Recent favorable experiences with autologous transplantation for severe aplastic anemia using privately stored CBU, suggested a possible utility. While private banking is difficult to justify statistically or empirically, there may exist rare cases where autologous transplant of stored umbilical CBU could be beneficial. The reality of privately banked CBU and the possibility for future discovery of additional indications for autologous cord blood transplant, motivated us to re‐examine our attitudes towards private cord blood banking. Pediatr Blood Cancer © 2011 Wiley...

Hepatitis Associated Aplastic Anemia: A review

Virology Journal — Mon, 28 Feb 2011 00:00:00 +0100

Hepatitis-associated aplastic anemia (HAAA) is an uncommon but distinct variant of aplastic anemia in which pancytopenia appears two to three months after an acute attack of hepatitis. HAAA occurs most frequently in young male children and is lethal if leave untreated. The etiology of this syndrome is proposed to be attributed to various hepatitis and non hepatitis viruses. Several hepatitis viruses such as HAV, HBV, HCV, HDV, HEV and HGV have been associated with this set of symptoms. Viruses other than the hepatitis viruses such as parvovirus B19, Cytomegalovirus, Epstein bar virus, Transfusion Transmitted virus (TTV) and non-A-E hepatitis virus (unknown viruses) has also been documented to develop the syndrome. Considerable evidences including the clinical features, severe imbalance of ...

Ticlopidine: Aplastic anaemia and neuroborreliosis: case report

Reactions — Sun, 27 Feb 2011 18:11:29 +0100

(Source: Reactions)

Paroxysmal nocturnal hemoglobinuria (PNH): higher sensitivity and validity in diagnosis and serial monitoring by flow cytometric analysis of reticulocytes

Annals of Hematology — Fri, 25 Feb 2011 17:05:44 +0100

In conclusion, a screening panel of at least two different GPI-AP markers on granulocytes, erythrocytes, and reticulocytes provides a simple and rapid method to detect even small GPI-deficient populations. Among the markers in our panel, CD58 and CD59 on reticulocytes, CD24/66b, and eventually FLAER on granulocytes as well as CD14 on monocytes were most effective for flow cytometric diagnosis of GPI deficiency. Content Type Journal ArticlePages 1-13DOI 10.1007/s00277-011-1177-4Authors Britta Höchsmann, DRK-Blood Donor Service, Baden-Württemberg-Hessia, Institute for Clinical Transfusion Medicine and Immunogenetics Ulm, Helmholtzstraße 10, 89081 Ulm, GermanyMarkus Rojewski, Institute of Transfusion Medicine, University of Ulm, Ulm, GermanyHubert Schrezenmeier, DRK-Blood Donor S...

Transient aplastic anemia in down's syndrome- a rare association.

European Journal of Medical Genetics — Thu, 24 Feb 2011 00:00:00 +0100

Authors: Gathwala G, Dalal P, Dalal JS, Choudhry O The association of Down's syndrome with aplastic anemia is extremely rare with only six such cases reported in world literature. Herein, we report a child of Down's syndrome with pancytopenia and hypocellular marrow. There was associated hypothyroidism and the pancytopenia resolved with thyroxine treatment. The child made uneventful recovery. PMID: 21354344 [PubMed - as supplied by publisher] (Source: European Journal of Medical Genetics)

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Relapse of aplastic anemia responsive to sirolimus combined with cyclosporine

Pediatric Blood and Cancer — Fri, 11 Feb 2011 00:00:00 +0100

We report two cases of AA which relapsed after CsA and ATG plus CsA, respectively. They achieved transfusion independence after retreatment with sirolimus in combination with a CsA. Pediatr Blood Cancer © 2011 Wiley‐Liss, Inc. (Source: Pediatric Blood and Cancer)

Megaloblastic pancytopenia vis‐à‐vis non‐megaloblastic pancytopenia: is mean platelet volume useful discriminating indicator

Clinical and Laboratory Haematology — Thu, 10 Feb 2011 00:00:00 +0100

Conclusion: MPV has limited sensitivity and specificity to discriminate between megaloblastic and non‐megaloblastic pancytopenia. Pancytopenia due to aplastic/hypocellular marrow and acute leukaemia has significantly lower MPV than megaloblastic group while other pancytopenic cases do not show any statistical difference in MPV from megaloblastic pancytopenia. (Source: Clinical and Laboratory Haematology)

Megaloblastic pancytopenia vis-à-vis non-megaloblastic pancytopenia: is mean platelet volume useful discriminating indicator.

International Journal of Laboratory Hematology — Thu, 10 Feb 2011 00:00:00 +0100

Conclusion: MPV has limited sensitivity and specificity to discriminate between megaloblastic and non-megaloblastic pancytopenia. Pancytopenia due to aplastic/hypocellular marrow and acute leukaemia has significantly lower MPV than megaloblastic group while other pancytopenic cases do not show any statistical difference in MPV from megaloblastic pancytopenia. PMID: 21310007 [PubMed - as supplied by publisher] (Source: International Journal of Laboratory Hematology)

Incidence of adult acquired severe aplastic anemia was not increased in Shanghai, China

Annals of Hematology — Thu, 03 Feb 2011 07:12:45 +0100

Content Type Journal ArticlePages 1-2DOI 10.1007/s00277-011-1168-5Authors Rong Fan, Hematology Department of Huashan Hospital, Fudan University, Shanghai, ChinaWei Wang, Hematology Department of Huashan Hospital, Fudan University, Shanghai, ChinaXiao-Qin Wang, Hematology Department of Huashan Hospital, Fudan University, Shanghai, ChinaGuo-Wei Lin, Hematology Department of Huashan Hospital, Fudan University, Shanghai, China Journal Annals of HematologyOnline ISSN 1432-0584Print ISSN 0939-5555 (Source: Annals of Hematology)

A case of Klinefelter syndrome with aplastic anemia.

International Journal of Hematology — Tue, 01 Feb 2011 00:00:00 +0100

Authors: Xu C, Zhang CY, Chen N, Sun X, Xiao Y, Gao L, Zhao JJ In this manuscript, we report the first patient who suffered from Klinefelter syndrome with aplastic anemia and hyperprolactinemia. PMID: 21293954 [PubMed - in process] (Source: International Journal of Hematology)

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Danazol as first-line therapy for aplastic anemia

Annals of Hematology — Fri, 28 Jan 2011 20:30:09 +0100

Abstract Immunosuppressive therapy (IST) with anti-thymocyte globulin (ATG) plus cyclosporine A (CsA) is the standard treatment for aplastic anemia (AA) patients not eligible for allogeneic hematopoietic stem cell transplantation (HSCT). In the absence of ATG + CsA, androgens continue to be a treatment option. We documented the clinical evolution of AA patients treated with danazol instead of ATG + CsA. AA patients lacking both, human leukocyte antigen-matched donor and access to IST, were treated with danazol and modern support therapy and compared with those receiving a HSCT. Overall survival (OS), response rates, and death risk odds were calculated. Fifty AA patients were studied. Thirteen received a HSCT and 37 danazol and support therapy. Median daily dose of danazol ...

Predicting response to immunosuppressive therapy in childhood aplastic anemia.

Haematologica — Thu, 27 Jan 2011 00:00:00 +0100

In conclusion, pretreatment clinical and laboratory findings influence response to therapy. The finding that response rate worsens with increasing interval between diagnosis and treatment highlights the importance of prompt immunosuppressive therapy for patients with aplastic anemia. PMID: 21273269 [PubMed - as supplied by publisher] (Source: Haematologica)

Oral Iron Chelator Deferasirox in the Treatment of Secondary Hemochromatosis following Bone Marrow Transplantation in a Patient with Severe Aplastic Anemia.

Acta Haematologica — Tue, 25 Jan 2011 00:00:00 +0100

Authors: Quarta A, Melpignano A, Quarta G Iron overload sometimes complicates the clinical course of bone marrow transplantation and can cause damage to liver and heart function. A patient with post-transplantation secondary hemochromatosis was treated with deferasirox, which not only normalized ferritin levels, but also reduced hepatic iron to normal values as measured by biosusceptometry with a superconducting quantum interference device. Side effects were minimal. The use of deferasirox to reduce post-transplant iron burden merits evaluation in a larger patient population. PMID: 21266801 [PubMed - as supplied by publisher] (Source: Acta Haematologica)

Focal sparing of iron and fat in liver tissue in patients with hemosiderosis: diagnosis with combination of R2* relaxometry and proton density fat fraction calculation by MRI.

Diagnostic and Interventional Radiology : The Turkish Society of Radiology — Tue, 25 Jan 2011 00:00:00 +0100

CONCLUSION: Focal fat and iron sparing in patients with liver siderosis can mimic a lesion. Quantitative MRI techniques can help to characterize abnormal signal changes in segment 4 of the liver in patients with hepatic iron overload and can eliminate the need for biopsy of pseudolesions. PMID: 21267943 [PubMed - as supplied by publisher] (Source: Diagnostic and Interventional Radiology : The Turkish Society of Radiology)

Oral Iron Chelator Deferasirox in the Treatment of Secondary Hemochromatosis following Bone Marrow Transplantation in a Patient with Severe Aplastic Anemia

Karger Publishers — Mon, 24 Jan 2011 14:54:48 +0100

Acta Haematol 2011;125:219–221 (DOI:10.1159/000322802) (Source: Karger Publishers)

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Hepatitis G Virus associated aplastic anemia: A recent case from Pakistan

Virology Journal — Fri, 21 Jan 2011 00:00:00 +0100

Conclusion: HAAA is an uncommon but severe condition, which may occur following idiopathic cases of acute hepatitis. Our finding suggests the involvement of HGV in the development of aplastic anemia. In patients presenting with pancytopenia after an episode of acute hepatitis, the definitive diagnosis should be considered and confirmed by RT-PCR and if possible by bone marrow biopsy. (Source: Virology Journal)

High prevalence of oral human papillomavirus infection in Fanconi’s anemia patients

Oral Diseases — Wed, 19 Jan 2011 00:00:00 +0100

Conclusion: Patients without oral malignant lesions submitted to HSCT, have high prevalence of oral HPV. HPV screening and close follow up should be considered in these patients. (Source: Oral Diseases)

Dapsone‐induced methemoglobinemia

Cancer — Tue, 18 Jan 2011 00:00:00 +0100

CONCLUSIONS:Methemoglobinemia occurred in almost 20% of pediatric oncology patients receiving dapsone for PCP prophylaxis. Higher dapsone dosing is associated with increased risk. A cross‐sectionally acquired cytochrome b5 reductase enzyme activity level was not associated with methemoglobinemia risk. Studies are needed to define biologic correlates of methemoglobinemia and evaluate lower dapsone doses for PCP prophylaxis. Cancer 2011. © 2011 American Cancer Society. (Source: Cancer)

Circulating myeloid dendritic cells are increased in individuals with severe aplastic anemia.

International Journal of Hematology — Fri, 14 Jan 2011 00:00:00 +0100

The objectives of the study were to investigate the number of myeloid dendritic cells (mDC) and plasmacytoid dendritic cells (pDC) present in peripheral blood mononuclear cells (PBMC) from severe aplastic anemia (SAA) patients before and after intensive immunosuppressive therapy (IST) and to assess the expression of co-stimulatory molecules (CD80, CD86, and CD40) expressed by dendritic cells (DC) from SAA patients. The quantities of mDC and pDC and ratios of mDC to pDC in PBMC were measured in 38 SAA patients at active phase, 19 patients at recovery phase, and 17 normal controls. The surface expression of CD80, CD86, and CD40 on DCs and B lymphocytes was analyzed in 16 SAA patients and 15 normal controls. The percentages of mDC and the ratio of mDC:pDC of SAA patients at active phase incre...