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Recent advances in the understanding of iron metabolism and iron-related diseases.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
PMID: 19907143 [PubMed - in process] (Source: Acta Haematologica)
Source: Acta Haematologica - November 13, 2009 Category: Hematology Authors: Hoffbrand AV Tags: Acta Haematol Source Type: journals

The role of hepcidin in iron metabolism.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Hepcidin is the central regulator of systemic iron homeostasis. Dysregulation of hepcidin production results in a variety of iron disorders. Hepcidin deficiency is the cause of iron overload in hereditary hemochromatosis, iron-loading anemias, and hepatitis C. Hepcidin excess is associated with anemia of inflammation, chronic kidney disease and iron-refractory iron deficiency anemia. Diagnostic and therapeutic applications of this new knowledge are beginning to emerge. Dr. Ernest Beutler played a significant role in advancing our understanding of the function of hepcidin. This review is dedicated to his memory. PMI...
Source: Acta Haematologica - November 13, 2009 Category: Hematology Authors: Nemeth E, Ganz T Tags: Acta Haematol Source Type: journals

Role of matriptase-2 (TMPRSS6) in iron metabolism.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Iron, an essential element for life, is regulated primarily at the level of uptake, storage, and transport in order to maintain sufficient availability for normal physiology. The key protein in iron homeostasis is a 25-amino-acid peptide, hepcidin, which modulates the amount of iron in the circulation by binding and promoting the degradation of the iron exporter ferroportin. Given the central importance of hepcidin, recent studies have focused on how iron is sensed and how the iron signal is transmitted to hepcidin. Mutations in a type II serine protease, matriptase-2/TMPRSS6, were recently identified to be associated ...
Source: Acta Haematologica - November 13, 2009 Category: Hematology Authors: Lee P Tags: Acta Haematol Source Type: journals

Iron deficiency, Helicobacter infection and gastritis.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Despite elegant regulatory mechanisms, iron deficiency anemia (IDA) remains one of the most common nutritional deficiencies of mankind. Iron deficiency is the result of an interplay between increased host requirements, limited external supply, and increased blood loss. When related to increased physiologic needs associated with normal development, iron deficiency is designated physiologic or nutritional. By contrast, pathological iron deficiency, with the exception of gross menorrhagia, is most often the result of gastrointestinal disease associated with abnormal blood loss or malabsorption. If gastroenterologic evalua...
Source: Acta Haematologica - November 13, 2009 Category: Hematology Authors: Hershko C, Ronson A Tags: Acta Haematol Source Type: journals

Anemia of chronic disease (anemia of inflammation).Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Mild-to-moderate anemia often develops in the setting of acute or chronic immune activation and is termed anemia of chronic disease (ACD) or anemia of inflammation. Anemia of chronic disease is the second most common type of anemia (after anemia of iron deficiency) and results in increased morbidity and mortality of the underlying disease. Anemia of chronic disease is mediated by inflammatory cytokines and is characterized by low serum iron (hypoferremia) and often increased reticuloendothelial stores of iron. Hepcidin is the master regulator of iron homeostasis and its synthesis is inhibited by iron deficiency and sti...
Source: Acta Haematologica - November 13, 2009 Category: Hematology Authors: Agarwal N, Prchal JT Tags: Acta Haematol Source Type: journals

The anemia of heart failure.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Anemia is common in congestive heart failure (CHF) and is associated with an increased mortality and morbidity. The most likely causes of anemia are chronic kidney disease (CKD) and excessive cytokine production, both of which can cause depression of erythropoietin (EPO) production and bone marrow activity. The cytokines also induce iron deficiency by both reducing gastrointestinal iron absorption and iron release from iron stores located in the macrophages and hepatocytes. Iron deficiency can cause thrombocytosis which might also contribute to cardiovascular complications in both CHF and CKD and is partially reversibl...
Source: Acta Haematologica - November 13, 2009 Category: Hematology Authors: Silverberg DS, Wexler D, Palazzuoli A, Iaina A, Schwartz D Tags: Acta Haematol Source Type: journals

Mitochondrial iron metabolism and sideroblastic anemia.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Sideroblastic anemias are a heterogeneous group of disorders, characterized by mitochondrial iron overload in developing red blood cells. The unifying characteristic of all sideroblastic anemias is the ring sideroblast, which is a pathological erythroid precursor containing excessive deposits of non-heme iron in mitochondria with perinuclear distribution creating a ring appearance. Sideroblastic anemias may be hereditary or acquired. Hereditary sideroblastic anemias are caused by defects in genes present on the X chromosome (mutations in the ALAS2, ABCB7, or GRLX5 gene), genes on autosomal chromosomes, or mitochondrial...
Source: Acta Haematologica - November 13, 2009 Category: Hematology Authors: Sheftel AD, Richardson DR, Prchal J, Ponka P Tags: Acta Haematol Source Type: journals

The natural history of untreated HFE-related hemochromatosis.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Hemochromatosis has generally been considered to be a genetic disease in which progressive iron accumulation over many years can lead to cirrhosis of the liver, hepatocellular carcinoma, diabetes, cardiomyopathy, and arthropathy. Iron depletion by phlebotomy has been the recommended therapy although a randomized trial of phlebotomy versus no treatment has never been reported. Since the discovery of the HFE gene in 1996, it has been possible to predict the risk of developing iron overload by a simple blood test to detect C282Y homozygotes of the HFE gene. The application of the hemochromatosis genetic test in large popu...
Source: Acta Haematologica - November 13, 2009 Category: Hematology Authors: Adams PC Tags: Acta Haematol Source Type: journals

Rare types of genetic hemochromatosis.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Most types of genetic hemochromatosis are due to mutations in the HFE gene, although similar iron overload and organ damage can also result from mutations in genes other than HFE in rare types of hemochromatosis. Non-HFE hemochromatoses have been divided into two subgroups with distinctive features. The first includes juvenile and TFR2-related hemochromatoses that, similar to HFE hemochromatosis, show recessive inheritance, increased transferrin saturation, iron storage in hepatocytes and responsiveness to phlebotomy. Disorders in this subgroup, although differing regarding the severity of iron overload and/or the age ...
Source: Acta Haematologica - November 13, 2009 Category: Hematology Authors: Camaschella C, Poggiali E Tags: Acta Haematol Source Type: journals

Role of T2* magnetic resonance in monitoring iron chelation therapy.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The monitoring of chelation therapy is a very important part of the management of transfusion-dependent patients. Classical methods of monitoring iron loading are either unreliable or unable to detect important myocardial siderosis which can predispose to the development of cardiac complications such as heart failure. The development of the T2* technique using cardiovascular magnetic resonance has allowed clinicians to have a reliable method for measuring cardiac iron to guide chelation therapy. T2* can identify early those patients who are at risk of developing cardiac complications, enabling personalised, tailored th...
Source: Acta Haematologica - November 13, 2009 Category: Hematology Authors: Carpenter JP, Pennell DJ Tags: Acta Haematol Source Type: journals

Deferiprone chelation therapy for thalassemia major.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Iron overload is one of the major causes of morbidity in patients with thalassemia major. Deferiprone (DFP), an orally active iron chelator, emerged from an extensive search for new drugs to treat iron overload. Comparative studies have shown that at comparable doses the efficacy of DFP in removing body iron is similar to that of desferoxamine (DFO). In retrospective and prospective studies, DFP monotherapy was significantly more effective than DFO in the treatment of myocardial siderosis in thalassemia major. DFP can be used in combination with DFO in the management of severe iron overload. This chelation regimen is t...
Source: Acta Haematologica - November 13, 2009 Category: Hematology Authors: Galanello R, Campus S Tags: Acta Haematol Source Type: journals

Deferasirox (Exjade) for the treatment of iron overload.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Deferasirox is a once-daily oral iron chelator with established dose-dependent efficacy in both adult and pediatric patients with transfusional iron overload. The clinical development program has demonstrated the efficacy of deferasirox for up to 4.5 years of treatment in patients with various underlying anemias, including beta-thalassemia, myelodysplastic syndromes, sickle cell disease, aplastic anemia, and other rare anemias. In addition to reducing key indicators of total body iron levels (serum ferritin, liver iron concentration, and toxic labile plasma iron), deferasirox has also demonstrated the ability to remove...
Source: Acta Haematologica - November 13, 2009 Category: Hematology Authors: Cappellini MD, Taher A Tags: Acta Haematol Source Type: journals

Iron metabolism and iron chelation in sickle cell disease.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
This review highlights recent advances in iron metabolism that are relevant to sickle cell disease (SCD). SCD is a common hemoglobinopathy that results in chronic inflammation. Improved understanding of how iron metabolism is controlled by proteins such as hepcidin, ferroportin, hypoxia-inducible factor 1, and growth differentiation factor 15 have revealed how they are involved in the organ toxicity of SCD. SCD patients have lower levels of non-transferrin-bound iron (NTBI) relative to other hemoglobinopathies, such as thalassemia. Care for SCD now commonly uses transfusion that results in iron overload and necessitate...
Source: Acta Haematologica - November 13, 2009 Category: Hematology Authors: Walter PB, Harmatz P, Vichinsky E Tags: Acta Haematol Source Type: journals

Activity of Thalidomide and Lenalidomide in Mantle Cell Lymphoma.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In conclusion, our data support a role for immunomodulatory drugs in the treatment of MCL. PMID: 19907157 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - November 12, 2009 Category: Hematology Authors: Richardson SJ, Eve HE, Copplestone JA, Dyer MJ, Rule SA Tags: Acta Haematol Source Type: journals

Fragmentation of Human Erythrocyte Actin following Exposure to Hypoxia.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In conclusion, these results indicate that after exposure to hypoxia, RBCs display a modification of their actin and cytoskeleton instability. PMID: 19907156 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - November 10, 2009 Category: Hematology Authors: Risso A, Santamaria B, Pistarino E, Cosulich ME, Pompach P, Bezouska K, Antonutto G Tags: Acta Haematol Source Type: journals

The Growth Factor Independence-1 (Gfi1) Is Overexpressed in Chronic Myelogenous Leukemia.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The activation of ABL tyrosine kinase in BCR/ABL-positive chronic myelogenous leukemia (CML) leads to a diversity of biological changes related to the pathogenesis of the disease. In CML patients, we determined the expression of growth factor independence-1 (Gfi1), a transcription repressor with weak oncogenic activity. Our data demonstrated that the Gfi1 mRNA level in both the mononuclear cells and purified CD34(+) cells from CML were significantly higher as measured by quantitative real-time PCR. Using flow cytometry and Western blot, we also showed that the Gfi1 protein content was increased in CML CD34(+) cells. Th...
Source: Acta Haematologica - November 2, 2009 Category: Hematology Authors: Huang M, Hu Z, Chang W, Ou D, Zhou J, Zhang Y Tags: Acta Haematol Source Type: journals

Hepcidin Serum Levels and Resistance to Recombinant Human Erythropoietin Therapy in Haemodialysis Patients.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
PMID: 19887781 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - October 30, 2009 Category: Hematology Authors: Costa E, Swinkels DW, Laarakkers CM, Rocha-Pereira P, Rocha S, Reis F, Teixeira F, Miranda V, do Sameiro Faria M, Loureiro A, Quintanilha A, Belo L, Santos-Silva A Tags: Acta Haematol Source Type: journals

Sustained Expression of GRAIL during Hematopoiesis Results in Dysregulated Differentiation.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Conclusion: These data highlight a role for GRAIL during hematopoiesis and emphasize the importance of studying posttranslational effects to complement our current understanding of the transcriptional regulation of hematopoiesis. PMID: 19887782 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - October 30, 2009 Category: Hematology Authors: Mackenzie DA, Seroogy CM Tags: Acta Haematol Source Type: journals

Prognostic Factors of Treatment Outcomes in Patients with Granulocytic Sarcoma.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Conclusion: Early diagnosis with biopsy and early chemotherapy can improve survival outcome. Local radiation or surgery can improve symptoms but does not influence survival outcomes. PMID: 19887783 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - October 30, 2009 Category: Hematology Authors: Lan TY, Lin DT, Tien HF, Yang RS, Chen CY, Wu K Tags: Acta Haematol Source Type: journals

Associations between Enhanced Fetal Hemoglobin Levels and Ineffective Reticulocyte Production in Diabetics.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
PMID: 19887784 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - October 30, 2009 Category: Hematology Authors: Lee MH, Kim JH, Im MW, Choi JW Tags: Acta Haematol Source Type: journals

Second Allogeneic Stem Cell Transplantation in Myeloid Malignancies.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In conclusion, a second SCT offers the chance of stable remission for some patients relapsing with a myeloid malignancy after a first allo-SCT, although high treatment-related mortality and relapse rates remain a problem. Efforts should concentrate on an optimization of conditioning strategies, immunosuppression and post-transplant surveillance for this specific situation. PMID: 19887774 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - October 29, 2009 Category: Hematology Authors: Hartwig M, Ocheni S, Asenova S, Wiedemann B, Zabelina T, Ayuk F, Kabisch H, Erttmann R, Kröger N, Zander AR, Bacher U Tags: Acta Haematol Source Type: journals

Utility of the Follicular Lymphoma International Prognostic Index and the International Prognostic Index in Assessing Prognosis and Predicting First-Line Treatment Efficacy in Follicular Lymphoma Patients.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Conclusion: Our data support the prognostic value of both IPI and FLIPI, with FLIPI demonstrating a more convenient patient distribution among risk classes. A low FLIPI score was also associated with a longer TTF. This might partially contribute to a more favourable prognosis. PMID: 19887775 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - October 29, 2009 Category: Hematology Authors: Formica V, Norman AR, Cunningham D, Wotherspoon A, Oates J, Chong G Tags: Acta Haematol Source Type: journals

Clinical Features and Survival Outcomes in Patients with Multiple Myeloma: Analysis of Web-Based Data from the Korean Myeloma Registry.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Conclusions: We demonstrated associations between survival outcomes and treatment modalities as well as baseline disease characteristics in a registry of multiple myeloma patients using a web-based data analysis. PMID: 19887776 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - October 29, 2009 Category: Hematology Authors: Kim SJ, Kim K, Kim BS, Jo DY, Kang HJ, Kim JS, Mun YC, Kim CS, Sohn SK, Eom HS, Kwak JY, Shim H, Yoon HJ, Jin JY, Min CK, Shin H, Won JH, Lee JJ, Kwon JH, Joo YD, Do YR, Kim SH, Oh S, Suh C, Lee J, Yoon SS, Kim MK, Bang SM, Ryoo HM, Kim BS, Kim H, Kim HJ, Tags: Acta Haematol Source Type: journals

Treatment-Related Burkitt's Lymphoma: Literature Review and Case Report of Successful Treatment with Rituximab Monotherapy.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We report a rare case of an elderly patient with radiotherapy-related BL. A 71-year-old Japanese man, who had a past history of oropharyngeal cancer treated with local irradiation 15 years ago, presented with a left mandibular mass in December 2004. A partial mandibulectomy disclosed pathological features consistent with BL. Although the patient was initially treated with intensive chemotherapy, the development of complications precluded further anticancer drug treatment. Rituximab was administered once weekly for 5 consecutive weeks, with resolution of the mandibular mass. He remained in remission without further lymphoma...
Source: Acta Haematologica - October 29, 2009 Category: Hematology Authors: Nagasaki A, Yamanoha A, Okudaira T, Miyagi T, Takasu N Tags: Acta Haematol Source Type: journals

Successful Treatment of Hepatic Encephalopathy in a Patient with Acute Lymphoblastic Leukemia.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We describe a case of a 32-year-old woman with severe icterus, thrombocytopenia, neutropenia and hepatosplenomegaly. ALL was diagnosed. One day after admission, she developed hepatic encephalopathy with ammonia concentrations in plasma >100 mumol/l. Hepatic infiltration was presumed and chemotherapy was initiated immediately which led to resolution of hepatic encephalopathy and complete hematological remission. Clinicians should be aware of unusual presentations of ALL and difficulties for the application of chemotherapy in patients with liver failure. PMID: 19887778 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - October 29, 2009 Category: Hematology Authors: Dada R, Wilop S, Jost E, Galm O, Gassler N, Osieka R Tags: Acta Haematol Source Type: journals

Anagrelide Treatment in Early Pregnancy in a Patient with JAK2V617F-Positive Essential Thrombocythemia: Case Report and Literature Review.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
PMID: 19887779 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - October 29, 2009 Category: Hematology Authors: Sobas MA, Pérez Encinas MM, Rabuñal Martinez MJ, Quinteiro García C, Bello López JL Tags: Acta Haematol Source Type: journals

Identification of a Novel Mutation in the L Ferritin Iron-Responsive Element Causing Hereditary Hyperferritinemia-Cataract Syndrome.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
PMID: 19887780 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - October 29, 2009 Category: Hematology Authors: Messa E, Pellegrino RM, Palmieri A, Carturan S, Cilloni D, Saglio G, Roetto A Tags: Acta Haematol Source Type: journals

Absence of the V617F JAK2 Mutation in the Lymphoid Compartment in a Patient with Essential Thrombo- cythemia and B-Chronic Lymphocytic Leukemia and in Two Relatives with Lymphoproliferative Disorders.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Conclusion: Our results seem to confirm that CLL cases are negative for JAK2(V617F) mutation in B- and T-lymphocyte populations.Presence of JAK2(V617F) mutation in subjects without myeloproliferative diseases could indicate an increased risk of a future myeloproliferative neoplasm development. PMID: 19816006 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - October 6, 2009 Category: Hematology Authors: Musolino C, Allegra A, Penna G, Centorrino R, Cuzzola M, D'Angelo A, Iacopino P, Alonci A Tags: Acta Haematol Source Type: journals

Prenatal Diagnosis of Hemoglobin Bart's Hydrops Fetalis with Gap-PCR System.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
PMID: 19816007 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - October 6, 2009 Category: Hematology Authors: Li DZ Tags: Acta Haematol Source Type: journals

Response to Dr. Dong-Zhi Li.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
PMID: 19816008 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - October 6, 2009 Category: Hematology Authors: Karnpean R, Fucharoen G, Fucharoen S, Sae-Ung N, Sanchaisuriya K, Ratanasiri T Tags: Acta Haematol Source Type: journals

Arsenic Trioxide in a Hemodialytic Patient with Acute Promyelocytic Leukemia.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
PMID: 19816009 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - October 6, 2009 Category: Hematology Authors: Yamamoto Y, Sasaki M, Oshimi K, Sugimoto K Tags: Acta Haematol Source Type: journals

Effect of L-Asparaginase Combined with Vincristine and Prednisolone on Acute Myeloblastic Leukemia (M0) Associated with Non-Hodgkin Lymphoma.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
A 66-year-old Japanese woman was referred to us because of severe anemia and fever and presented at our hospital. She was eventually diagnosed as having acute myeloblastic leukemia (AML; M0) with non-Hodgkin lymphoma (NHL). We investigated the therapeutic efficacy of L-asparaginase (L-Asp), vincristine and prednisolone for both her AML and NHL. Asparagine synthetase (AS) activity in her AML blast cells was undetectable. A lymph node biopsy specimen revealed NHL of the marginal zone B cell type. Complete remission (CR) of AML and NHL was achieved. CR of the AML lasted for 18 months without further consolidation therapy....
Source: Acta Haematologica - October 6, 2009 Category: Hematology Authors: Horikoshi A, Takei K, Iriyama N, Uenogawa K, Ishizuka H, Shiraiwa H, Hosokawa Y, Sawada S, Kitoh T Tags: Acta Haematol Source Type: journals

Self-Monitoring of Oral Anticoagulation Therapy in Children.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In conclusion, home INR self-monitoring is accurate for children requiring long-term oral anticoagulation therapy. Our data suggest that INR self-monitoring with the newer CoaguChek XS is more accurate than with the older CoaguChek S monitor. PMID: 19816011 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - October 6, 2009 Category: Hematology Authors: Paioni P, Kroiss S, Kägi E, Bergsträsser E, Fasnacht M, Bauersfeld U, Schmugge M, Albisetti M Tags: Acta Haematol Source Type: journals

Elevated Circulating Stromal-Derived Factor-1 Levels in Sickle Cell Disease.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In this study we determined serum SDF-1 levels in consecutive adult sickle cell patients during the clinically asymptomatic state as well as during painful crisis. Serum SDF-1 levels were significantly elevated in HbSS/HbSbeta(0)-thalassaemia patients [n = 42; 5,177 pg/ml (2,438-7,246)] compared to HbSC/HbSbeta(+)-thalassaemia patients [n = 16; 2,405 pg/ml (1,365-3,047)] and healthy HbAA controls [n = 45; 2,894 pg/ml (2,577-3,334)] (p = 0.001). No significant increments were observed during painful crisis (n = 40). SDF-1 levels were significantly higher in SCD patients with pulmonary hypertension (PHT) compared to patients...
Source: Acta Haematologica - October 6, 2009 Category: Hematology Authors: Landburg PP, Nur E, Maria N, Brandjes DP, Biemond BJ, Schnog JB, Duits AJ Tags: Acta Haematol Source Type: journals

Bortezomib and Liposomal Doxorubicin Are Highly Effective in Obtaining the Best Possible Response before Autologous Transplant for Multiple Myeloma.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
PMID: 19729887 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - August 28, 2009 Category: Hematology Authors: Buda G, Orciuolo E, Galimberti S, Cecconi N, Petrini M Tags: Acta Haematol Source Type: journals

MDR1 C3435T Polymorphism Indicates a Different Outcome in Advanced Multiple Myeloma.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
PMID: 19729888 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - August 28, 2009 Category: Hematology Authors: Buda G, Martino A, Maggini V, Orciuolo E, Galimberti S, Gentile M, Morabito F, Antognoni F, Brunori M, Barale R, Rossi AM, Petrini M Tags: Acta Haematol Source Type: journals

Wild-Type JAK2 Secondary Acute Erythroleukemia Developing after JAK2-V617F-Mutated Primary Myelofibrosis.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
A 54-year-old female patient developed acute erythroleukemia after an 8-year course of primary myelofibrosis. The latter harbors the JAK2-V617F mutation and was treated with hydroxyurea and anagrelide. A bone marrow trephine biopsy disclosed 2 morphologically distinct areas of chronic primary myelofibrosis and acute erythroleukemia. Microdissection and a separate molecular pathological analysis was performed. Although the activating JAK2-V617F mutation was not maintained in blasts of acute erythroleukemia, it was detectable in the chronic phase of primary myelofibrosis, indicating that this mutation did not play a role...
Source: Acta Haematologica - August 27, 2009 Category: Hematology Authors: Kreft A, Springer E, Lipka DB, Kirkpatrick CJ Tags: Acta Haematol Source Type: journals

Molecular Analysis of a Novel Case of Congenital Atransferrinemia.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
PMID: 19696475 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - August 19, 2009 Category: Hematology Authors: Chen C, Wen S, Tan X Tags: Acta Haematol Source Type: journals

Clonal Switch of Leukemic Myeloblasts after Chemotherapy in a Patient with Chronic Myeloproliferative Disorder.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
PMID: 19696476 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - August 19, 2009 Category: Hematology Authors: Hashino S, Fujisawa F, Takahata M, Izumiyama K, Yonezumi M, Chiba K, Kondo T, Suzuki S, Asaka M Tags: Acta Haematol Source Type: journals

DNMT3B 579G>T Promoter Polymorphism and the Risk for Idiopathic Thrombocytopenic Purpura in a Chinese Population.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
DNMT3B 579G>T Promoter Polymorphism and the Risk for Idiopathic Thrombocytopenic Purpura in a Chinese Population. Acta Haematol. 2009 Aug 20;122(1):31-35 Authors: Zhao H, Du W, Gu D, Wang D, Xue F, Ge J, Sui T, Yang R Epigenetics may influence the expression of numerous genes, which might contribute to autoimmune diseases. DNA methylation is mediated by DNA methyltransferases, especially DNA methyltransferase 3B (DNMT3B). Polymorphisms of the DNMT3B gene may influence DNMT3B activity on DNA methylation and increase the susceptibility to several diseases. The current study investigated the association between...
Source: Acta Haematologica - August 19, 2009 Category: Hematology Authors: Zhao H, Du W, Gu D, Wang D, Xue F, Ge J, Sui T, Yang R Tags: Acta Haematol Source Type: journals

Improvement in the Detection of alpha0- and Deletional alpha-Thalassemia by Real-Time PCR Combined with Dissociation Curve Analysis.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The prevailing cause of alpha-thalassemia in Southeast Asia is the presence of 3 deletion mutations in the alpha-globin genes (-SEA, -alpha(3.7) and -alpha(4.2)). Current detection methods include gap polymerase chain reaction (PCR), multiplex PCR and real-time PCR with SYBR Green 1 combined with dissociation curve analysis. To improve and simplify a previously published method that requires 4 separate reactions, a duplex PCR assay was designed to detect both the nondeletional and the -SEA alleles. This duplex PCR can successfully identify the nondeletional allele and both the -SEA carrier and homozygous genotypes. The...
Source: Acta Haematologica - August 14, 2009 Category: Hematology Authors: Liu J, Tang N, Liu Q, Wang L, Han H, Cai R, Wu X, Xiao B Tags: Acta Haematol Source Type: journals

Clinical Characteristics and Treatment Outcome of Adult Acute Lymphoblastic Leukemia with t(4;11)(q21;q23) Using a Modified Hyper-CVAD Regimen.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
PMID: 19672058 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - August 5, 2009 Category: Hematology Authors: Li Y, Zou D, Zhao Y, Wang J, Qiu L Tags: Acta Haematol Source Type: journals

Chronic Myelogenous Leukemia with the e6a2 BCR-ABL and Lacking Imatinib Response: Presentation of Two Cases.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The BCR-ABL fusion gene represents the hallmark of chronic myelogenous leukemia (CML) and is derived from a translocation between chromosome 9 and 22. The majority of CML patients have a breakpoint in the major BCR region of the BCR gene giving rise to e13a2 or e14a2 BCR-ABL transcripts. Occasionally, other BCR breakpoints occur. The current report describes two e6a2 CML patients with imatinib treatment failure and unusual disease progression. One patient was Philadelphia chromosome positive and one was Philadelphia chromosome negative with an atypical BCR-ABL rearrangement, ins (22;9). PMID: 19641300 [PubMed - as ...
Source: Acta Haematologica - July 28, 2009 Category: Hematology Authors: Vefring HK, Gruber FX, Wee L, Hovland R, Hjorth-Hansen H, Gedde Dahl T, Meyer P Tags: Acta Haematol Source Type: journals

Second-Generation Tyrosine Kinase Inhibitors in the Post-Transplant Period in Patients with Chronic Myeloid Leukemia or Philadelphia-Positive Acute Lymphoblastic Leukemia.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
PMID: 19602874 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - July 14, 2009 Category: Hematology Authors: Klyuchnikov E, Schafhausen P, Kröger N, Brummendorf TH, Osanmaz O, Asenova S, Zabelina T, Ocheni S, Ayuk F, Zander AR, Bacher U Tags: Acta Haematol Source Type: journals

Dimethyl Sulfoxide-Induced Toxicity in Cord Blood Stem Cell Transplantation: Report of Three Cases and Review of the Literature.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Umbilical cord blood transplantation using nonmyeloablative conditioning is currently considered by many as a valid potential alternative for any patient who requires an unrelated donor allograft and who is without a suitably matched and readily available volunteer. Dimethyl sulfoxide (DMSO) has been used for years as a cryoprotectant agent; it acts by penetrating the cell and binding water molecules and it has been described as harmless for the individual who receives it in limited amounts. In this paper, we describe 3 cases of DMSO-induced toxicities and briefly review the most common adverse reactions of the DMSO wh...
Source: Acta Haematologica - July 2, 2009 Category: Hematology Authors: Ruiz-Delgado GJ, Mancías-Guerra C, Tamez-Gómez EL, Rodríguez-Romo LN, López-Otero A, Hernández-Arizpe A, Gómez-Almaguer D, Ruiz-Argüelles GJ Tags: Acta Haematol Source Type: journals

A Pilot Beta-Thalassaemia Screening Program in the Albanian Population for a Health Planning Program.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In Albania, no definite national screening programme of beta-thalassaemia has yet been developed for carrier detection. Only limited information about the occurrence and the types of haemoglobin abnormalities is available. Thus, an educational and screening programme was carried out in one high school with a total of 217 young students from the coastal province of Lushnja in Albania. The pilot programme included a systematic sampling of whole saliva, DNA genomic extraction and the determination of defective beta-thalassaemia genes by reverse dot-blot hybridization with 22 probes specific for the Mediterranean populatio...
Source: Acta Haematologica - June 27, 2009 Category: Hematology Authors: Baghernajad-Salehi L, D'Apice MR, Babameto-Laku A, Biancolella M, Mitre A, Russo S, Di Daniele N, Sangiuolo F, Mokini V, Novelli G Tags: Acta Haematol Source Type: journals

Autologous Peripheral Blood Stem Cell Transplantation to Treat CHOP-Refractory Aggressive Subcutaneous Panniculitis-Like T Cell Lymphoma.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
PMID: 19556752 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - June 27, 2009 Category: Hematology Authors: Nakahashi H, Tsukamoto N, Yamane A, Saitoh T, Uchiumi H, Handa H, Karasawa M, Murakami H, Kojima M, Nojima Y Tags: Acta Haematol Source Type: journals

Laboratory diagnosis and molecular classification of von Willebrand disease.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
A complete set of laboratory investigations, including bleeding time, PFA-100 closure times, factor VIII (FVIII) coagulant activity (FVIII:C), von Willebrand factor (VWF) ristocetin cofactor (VWF:RCo), collagen binding (VWF:CB), antigen (VWF:Ag) and propeptide (VWFpp), ristocetin-induced platelet aggregation (RIPA), multimeric analysis of VWF and the response of FVIII:C and VWF parameters to desmopressin (DDAVP), is necessary to fully diagnose all variants of von Willebrand disease (VWD) and to discriminate between type 1 and type 2 and between severe VWD type 1 and type 3. The response to DDAVP of VWF parameters is no...
Source: Acta Haematologica - June 27, 2009 Category: Hematology Authors: Gadisseur A, Hermans C, Berneman Z, Schroyens W, Deckmyn H, Michiels JJ Tags: Acta Haematol Source Type: journals

Laboratory diagnosis and molecular basis of mild von Willebrand disease type 1.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Mild type 1 von Willebrand disease (VWD) is characterized by low to variable penetrance of bleeding, a high (increased) prevalence of blood group O, von Willebrand factor (VWF) values around and above 30% with normal ratios of VWF:ristocetin cofactor activity (RCo)/VWF:antigen (Ag), VWF:collagen binding (CB)/VWF:Ag and factor VIII (FVIII):coagulant activity (C)/VWF:Ag. Within this group of patients, the combination of the C1584 mutation and blood group O is rather frequent. Patients with mild VWD type 1 present good/normal responses of FVIII:C and VWF parameters to desmopressin (DDAVP). With the exclusion of dominant V...
Source: Acta Haematologica - June 27, 2009 Category: Hematology Authors: Michiels JJ, Berneman Z, Gadisseur A, van der Planken M, Schroyens W, van Vliet HH Tags: Acta Haematol Source Type: journals

C1584: effect on von Willebrand factor proteolysis and von Willebrand factor antigen levels.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The present review provides a clearer picture of the effect of C1584 on the level and properties of von Willebrand factor (VWF). The C1584 variant is associated with decreased VWF levels (especially in combination with blood group O) and VWF function, and with slightly enhanced VWF proteolysis, and there is evidence to support its association with enhanced VWF clearance. These properties help explain the enrichment for C1584 in mild type 1 VWD and the observed marked association of the variant and blood group O in this mild bleeding disorder. It has been proposed that the mildly enhanced VWF proteolysis associated with...
Source: Acta Haematologica - June 27, 2009 Category: Hematology Authors: Davies JA, Collins PW, Hathaway LS, Bowen DJ Tags: Acta Haematol Source Type: journals