Haematologica current issue

Hepatitis B reactivation prophylaxis in immune-suppressed patients

Torresin, A., Feasi, M., Cassola, G. — 2008-10-31

Risk of HBV liver disease in isolated antiHbc patients receiving immuno-chemotherapy for non Hodgkin lymphoma

Targhetta, C., Cabras, M.G., Angelucci, E. — 2008-10-31

Use of hydroxyurea from childhood to adult age in sickle cell disease: semen analysis

Lukusa, A.K., Vermylen, C. — 2008-10-31

Reply to: [Use of hydroxyurea from childhood to adult age in sickle cell disease: semen analysis. Haematologica 2008; 93:e67]

Berthaut, I., Girot, R., Mandelbaum, J. — 2008-10-31

Diamond-Blackfan anemia: a ribosomal puzzle

Dianzani, I., Loreni, F. — 2008-10-31

Diamond-Blackfan anemia is the first, and so far only known, inherited disease due to a defect in a structural ribosomal protein. In this perspective article, Drs. Dianzani and Loreni examine this ribosomal puzzle. See related articles on pages 1617 and 1627.

AB0-incompatible allogeneic hematopoietic stem cell transplantation

Worel, N., Kalhs, P. — 2008-10-31

Due to the fact that the HLA system is inherited independently of the blood group system, approximately 40 to 50% of all hematopoietic stem cell transplants are performed across the AB0-blood group barrier. In this perspective article, Drs. Worel and Kahls examine the features and outcome of AB0-incompatible allogeneic hematopoietic stem cell transplantation. See related paper on page 1686.

Hematopoietic stem cell transplantation: 40 years of continuous progress and evolution

Porta, F., Locatelli, F., Burgio, G. R. — 2008-10-31

Hematopoietic stem cell transplantation has represented one of the most innovative treatments of the last decades, as well as one of the most significant medical feats of human bio-solidarity. In this perspective article, Drs. Porta, Locatelli and Burgio analyze 40 years of continuous progress and evolution in the field.

Pulmonary aspergillosis in hematologic malignancies: lights and shadows

Pagano, L., Fianchi, L., Caira, M. — 2008-10-31

The population of patients with hematological disorders at risk for pulmonary aspergillosis is expanding. In this perspective article, Drs. Pagano, Fianchi, and Caira analyze the recent advances in this field.

Enhanced alternative splicing of the FLVCR1 gene in Diamond Blackfan anemia disrupts FLVCR1 expression and function that are critical for erythropoiesis

2008-10-31

This study recapitulates the Diamond-Blackfan anemia hematologic features of reduced erythropoiesis but normal myelopoiesis by disrupting FLVCR1 in human hematopoietic stem cells. See related perspective article on page 1601.

Study of the effects of proteasome inhibitors on ribosomal protein S19 (RPS19) mutants, identified in patients with Diamond-Blackfan anemia

2008-10-31

The findings of this study demonstrate an important role for the proteasomal degradation pathway in regulating the levels of expression and nucleolar localization of certain mutant RPS19 proteins in Diamond-Blackfan anemia. See related perspective article on page 1601.

Methylation of the suppressor of cytokine signaling 3 gene (SOCS3) in myeloproliferative disorders

2008-10-31

Suppressor of cytokine signaling 3 (SOCS3) is negative regulator of the JAK/STAT pathway. Methylation of this gene might, therefore, contribute to the pathogenesis of myeloproliferative disorders. In this study, SOCS3 promoter methylation was detected in about one third of patients with idiopathic myelofibrosis suggesting a possible role for SOCS3 methylation in this disorder.

Prognostic factors for thrombosis, myelofibrosis, and leukemia in essential thrombocythemia: a study of 605 patients

2008-10-31

The findings from this study on a large series of patients treated according to current clinical practice provide reassurance that essential thrombocythemia is an indolent disorder and affected patients have a long survival.

Anemia and survival in childhood acute lymphoblastic leukemia

2008-10-31

The inverse relationship between severity of anemia and survival found within specific subgroups of patients with childhood acute lymphoblastic leukemia suggests that very low hemoglobin levels at diagnosis are associated with more advanced disease in these subgroups.

Impact of genotype on survival of children with T-cell acute lymphoblastic leukemia treated according to the French protocol FRALLE-93: the effect of TLX3/HOX11L2 gene expression on outcome

2008-10-31

The findings of this study suggest that TLX3 gene expression is an independent risk factor predicting poor survival in childhood T-cell acute lymphoblastic leukemia.

Anthropometric characteristics and non-Hodgkin's lymphoma and multiple myeloma risk in the European Prospective Investigation into Cancer and Nutrition (EPIC)

2008-10-31

The incidences of non-Hodgkin’s lymphoma and multiple myeloma are increasing steadily. It has been hypothesized that this may be due, in part, to the parallel rising prevalence of obesity. This European Prospective Investigation into Cancer and Nutrition (EPIC) study supports an association between height and overall non-Hodgkin’s lymphoma and multiple myeloma among women.

Nonsense-mediated mRNA decay in the ADAMTS13 gene caused by a 29-nucleotide deletion

Garagiola, I., Valsecchi, C., Lavoretano, S., Oren, H., Bohm, M., Peyvandi, F. — 2008-10-31

This study demonstrates that two cases of severe ADAMTS13 deficiency are mechanistically caused by the association of two different gene defects acting at two different levels.

Impact of AB0-blood group incompatibility on the outcome of recipients of bone marrow transplants from unrelated donors in the Japan Marrow Donor Program

2008-10-31

The findings of this study indicate that major and minor AB0 incompatibility have specific effects on transplant-related mortality and acute graft-versus-host disease in recipients of bone marrow transplants from unrelated donors. See related perspective article on page 1605.

Hematopoietic stem cell transplantation for hemophagocytic lymphohistiocytosis: a retrospective analysis of data from the Italian Association of Pediatric Hematology Oncology (AIEOP)

2008-10-31

This study confirms that hematopoietic stem cell transplantation represents a curative treatment for a large proportion of patients with hemophagocytic lymphohistiocytosis, irrespective of the underlying genetic defect.

B-cell involvement in chronic graft-versus-host disease

Kapur, R., Ebeling, S., Hagenbeek, A. — 2008-10-31

This article addresses the pathogenesis of chronic graft-versus-host disease, which is not yet completely understood, with a special focus on the possible role of B cells.

Diagnosis and classification of myelodysplastic syndrome: International Working Group on Morphology of myelodysplastic syndrome (IWGM-MDS) consensus proposals for the definition and enumeration of myeloblasts and ring sideroblasts

2008-10-31

This article details the proposals of the IWGM-MDS for the definition of myeloblasts, promyelocytes and ring side-roblasts in patients with myelodysplastic syndrome.

Imatinib and leptomycin B are effective in overcoming imatinib-resistance due to Bcr-Abl amplification and clonal evolution but not due to Bcr-Abl kinase domain mutation

2008-10-31

The findings of this study indicate that the combination of imatinib and leptomycin B effectively induces cell death in imatinib-resistant Ba/F3 cells which display Bcr-Abl amplification or signs of clonal evolution.

Frequent reduction or absence of detection of the JAK2-mutated clone in JAK2V617F-positive patients within the first years of hydroxyurea therapy

2008-10-31

In this study, hydroxyurea treatment reduced the percentage of JAK2V617F mutant alleles by >30% in 13/25 patients with essential thrombocythemia or polycythemia vera within the first year after diagnosis.

Transcriptional upregulation of p21/WAF/Cip1 in myeloid leukemic blasts expressing AML1-ETO

2008-10-31

The increased expression of p21/WAF/Cip1 in primary leukemic blasts suggests that elevated p21/WAF/Cip1 levels may contribute to specific features observed in AML1-ETO positive leukemia.

An investigation into whether deletions in 9p reflect prognosis in adult precursor B-cell acute lymphoblastic leukemia: a multi-center study of 381 patients

2008-10-31

The findings of this study suggest that chromosomal abnormalities involving 9p may have a significant negative impact on survival in adult B-precursor acute lymphoblastic leukemia.

Mobilization of peripheral blood stem cells in myeloma with either pegfilgrastim or filgrastim following chemotherapy

2008-10-31

In this study on patients with multiple myeloma, peripheral blood stem cell mobilization after chemotherapy was feasible and similarly effective with pegfilgrastim and filgrastim.

Novel point mutation in a leucine-rich repeat of the GPIb{alpha} chain of the platelet von Willebrand factor receptor, GPIb/IX/V, resulting in an inherited dominant form of Bernard-Soulier syndrome affecting two unrelated families: the N41H variant

2008-10-31

This reports describes a new variant of heterozygous Bernard-Soulier syndrome with autosomal dominant inheritance.

Multiplex ligation-dependent probe amplification enhances molecular diagnosis of Diamond-Blackfan anemia due to RPS19 deficiency

2008-10-31

Immune-mediated pure red cell aplasia in renal transplant recipients

Zuber, J., Beldjord, K., Casadevall, N., Thervet, E., Legendre, C., Varet, B. — 2008-10-31

Nonsense mutations of the {alpha}-spectrin gene in hereditary pyropoikilocytosis

Tolpinrud, W., Maksimova, Y. D., Forget, B. G., Gallagher, P. G. — 2008-10-31

First case of {gamma}-thalassemia in association with a {beta}S allele: a pitfall in the neonatal screening for sickle cell disease

2008-10-31

Long term biweekly 1 mg oral vitamin B12 ensures normal hematological parameters, but does not correct all other markers of vitamin B12 deficiency. A study in patients with inherited vitamin B12 deficiency

Vakur Bor, M., Cetin, M., Aytac, S., Altay, C., Ueland, P. M., Nexo, E. — 2008-10-31

Monitoring for cytomegalovirus and Epstein-Barr virus infection in chronic lymphocytic leukemia patients receiving i.v. fludarabine-cyclophosphamide combination and alemtuzumab as consolidation therapy

2008-10-31

Erratum

2008-10-31