abstract：:The inverse of the difference between rates, called the 'number needed to treat' (NNT), was suggested 20 years ago as a good way to present the results of comparisons of success or failure under different therapies. Such comparisons usually arise in randomised controlled trials and meta-analysis. This article reviews ...

journal_title：British journal of haematology

authors： Hutton JL

更新日期：2009-06-01 00:00:00

abstract：:Shwachman-Diamond Syndrome (SDS) is a multi-system genetic disorder with bone marrow failure. SBDS, the gene associated with SDS, has been postulated to play a role in ribosome biogenesis and RNA processing, but its functions are still unknown. To study whether these pathways are interrupted when Sbds protein is lost,...

journal_title：British journal of haematology

authors： Rujkijyanont P,Adams SL,Beyene J,Dror Y

更新日期：2009-06-01 00:00:00

abstract：:Pulmonary hypertension (PH) in patients with sickle cell disease (SCD) is linked to intravascular haemolysis, impaired nitric oxide bioavailability, renal dysfunction, and early mortality. Asymmetric dimethylarginine (ADMA), an endogenous inhibitor of nitric oxide synthases (NOS), is associated with vascular disease i...

journal_title：British journal of haematology

authors： Kato GJ,Wang Z,Machado RF,Blackwelder WC,Taylor JG 6th,Hazen SL

更新日期：2009-05-01 00:00:00

abstract：:Steady-state haemoglobin (Hb) desaturation is a common finding in sickle cell anaemia (Hb SS) that could predispose to stroke by limiting oxygen delivery to the brain. To determine its association with the risk of overt stroke, we examined the relationship between daytime Hb saturation measured by pulse oximetry (SpO(...

journal_title：British journal of haematology

authors： Quinn CT,Variste J,Dowling MM

更新日期：2009-05-01 00:00:00

abstract：:Due to the development of neurological toxicity and resistance to methotrexate (MTX), other antifolates have been evaluated for its potential replacement in the treatment of childhood acute lymphoblastic leukaemia (ALL). Aminopterin (AMT) has been suggested to provide clinical advantages over MTX and other antifolates...

journal_title：British journal of haematology

authors： Kang MH,Harutyunyan N,Hall CP,Papa RA,Lock RB

更新日期：2009-05-01 00:00:00

abstract：:Mantle cell lymphoma (MCL) is an aggressive non-Hodgkin lymphoma with a poor prognosis following first relapse. We present a subgroup analysis of an open-label phase II trial investigating the efficacy and safety of lenalidomide in patients with relapsed or refractory MCL. Oral lenalidomide 25 mg was self-administered...

journal_title：British journal of haematology

authors： Habermann TM,Lossos IS,Justice G,Vose JM,Wiernik PH,McBride K,Wride K,Ervin-Haynes A,Takeshita K,Pietronigro D,Zeldis JB,Tuscano JM

更新日期：2009-05-01 00:00:00

abstract：:The potential role of the infused B cell subset after Hematopoietic Stem Cell Transplantation has not been yet studied. The present study analyzed the impact of B cells on transplant outcome in 254 patients who received a bone marrow graft from a human leucocyte antigen-identical sibling donor. The influence of B line...

journal_title：British journal of haematology

authors： Michonneau D,Peffault de Latour R,Porcher R,Robin M,Benbunan M,Rocha V,Ribaud P,Ferry C,Devergie A,Vanneaux V,Gluckman E,Marolleau JP,Socié G,Larghero J

更新日期：2009-04-01 00:00:00

abstract：:Total knee replacement (TKR) is a safe treatment for alleviating pain and restoring physical function in end-stage arthropathy of the knee. First reports of TKR in haemophiliacs date back to the mid-1970s, however detailed information on long-term outcome is scarce. This study evaluated factors influencing the outcome...

journal_title：British journal of haematology

authors： Solimeno LP,Mancuso ME,Pasta G,Santagostino E,Perfetto S,Mannucci PM

更新日期：2009-04-01 00:00:00

abstract：:Prior case-control studies reported that levels of the soluble form of the endothelial protein C receptor (sEPCR) were strongly controlled by the PROCR 6963A/G polymorphism and higher levels were a risk factor for venous thromboembolism (VTE). We sought to prospectively examine the association of sEPCR and the 6963A/G...

journal_title：British journal of haematology

authors： Yamagishi K,Cushman M,Heckbert SR,Tsai MY,Folsom AR

更新日期：2009-04-01 00:00:00

abstract：:This study assessed the recruitment to an acute myeloid leukaemia (AML) trial (AML15) in a single centre, evaluated whether outcome was influenced by trial entry and whether the trial population could be considered representative of all AML patients by retrospective comparison of patient characteristics, trial entry a...

journal_title：British journal of haematology

authors： Stevens JM,Macdougall F,Jenner M,Oakervee H,Cavenagh J,Lister AT

更新日期：2009-04-01 00:00:00

abstract：:Glucocorticoid (GC) effects are mediated by the glucocorticoid receptor (GR). Several studies have demonstrated that a lower number of receptors per cell were associated with poor GC response. The regulation of GR expression is complex; the levels of GR can be autologously regulated by its ligand and also by transcrip...

journal_title：British journal of haematology

authors： Sánchez-Vega B,Gandhi V

更新日期：2009-03-01 00:00:00

abstract：:Both the 2001 World Health Organisation (WHO) classification of haematopoietic neoplasms and the 2008 WHO classification revision include a distinctive diagnostic category, refractory anaemia with ring sideroblasts and thrombocytosis (RARS-T), to describe those rare patients who have both >or=15% ring sideroblasts and...

journal_title：British journal of haematology

authors： Wardrop D,Steensma DP

更新日期：2009-03-01 00:00:00

abstract：:This study looked for clonal diversity in patients with a myeloproliferative neoplasm associated with more than one acquired genetic lesion. A tyrosine kinase mutation and a cytogenetic lesion were present in the same clone in six of seven patients. By contrast, the genetic lesions were present in separate clones in a...

journal_title：British journal of haematology

authors： Beer PA,Jones AV,Bench AJ,Goday-Fernandez A,Boyd EM,Vaghela KJ,Erber WN,Odeh B,Wright C,McMullin MF,Cullis J,Huntly BJ,Harrison CN,Cross NC,Green AR

更新日期：2009-03-01 00:00:00

abstract：:Bone marrow angiogenesis is suggested to play a role in the pathogenesis of acute myeloid leukaemia (AML) and endothelial cells may mediate chemosensitivity. This study investigated in vitro endothelial effects of coculture of microvascular endothelial cells (MVEC) with AML cells derived from 33 consecutive AML patien...

journal_title：British journal of haematology

authors： Hatfield K,Øyan AM,Ersvaer E,Kalland KH,Lassalle P,Gjertsen BT,Bruserud Ø

更新日期：2009-01-01 00:00:00

abstract：:The addition of rituximab (R) to CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) chemotherapy was a milestone in the development of front-line therapy for diffuse large B-cell lymphoma (DLBCL). R-CHOP and equivalent rituximab-containing anthracycline-based regimens are now widely accepted as the standard...

journal_title：British journal of haematology

authors： Gisselbrecht C

更新日期：2008-12-01 00:00:00

abstract：:Gemtuzumab ozogamicin (GO) monotherapy is reported to yield a 20-30% response rate in advanced acute myeloid leukaemia (AML). This study examined the efficacy and tolerability of GO combined with cytarabine (GOCYT) in children with refractory/relapsed CD33(+) AML. Seventeen children received GO 3 mg/m(2) on days 1, 4 ...

journal_title：British journal of haematology

authors： Brethon B,Yakouben K,Oudot C,Boutard P,Bruno B,Jérome C,Nelken B,de Lumley L,Bertrand Y,Dalle JH,Chevret S,Leblanc T,Baruchel A

更新日期：2008-11-01 00:00:00

abstract：:The multimeric plasma protein von Willebrand factor (VWF) is regulated in size by its protease, ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13). Y1605-M1606 cleavage site mutations and single nucleotide polymorphisms (SNPs) in the VWF A1 and A2 domains were examined for alter...

journal_title：British journal of haematology

authors： Pruss CM,Notley CR,Hegadorn CA,O'Brien LA,Lillicrap D

更新日期：2008-11-01 00:00:00

abstract：:Both atypical haemolytic uraemic syndrome (aHUS) and the HELLP syndrome (haemolytic anaemia, elevated liver enzymes, and low platelets) are thrombotic microangiopathies characterized by microvascular endothelial activation, cell injury and thrombosis. aHUS is a disease of complement dysregulation, specifically a gain ...

journal_title：British journal of haematology

authors： Fang CJ,Richards A,Liszewski MK,Kavanagh D,Atkinson JP

更新日期：2008-11-01 00:00:00

abstract：:The inv(8)(p11q13) chromosomal abnormality, described in acute myeloid leukaemias (AML), fuses the histone acetyl-transferase (HAT) MYST3 (MOZ) gene with another HAT gene, NCOA2 (TIF2). We generated a transgenic zebrafish in which the MYST3/NCOA2 fusion gene was expressed under control of the spi1 promoter. An AML dev...

journal_title：British journal of haematology

authors： Zhuravleva J,Paggetti J,Martin L,Hammann A,Solary E,Bastie JN,Delva L

更新日期：2008-11-01 00:00:00

abstract：:Some case reports and a Japanese series suggest benefit from the use of rituximab in patients with intravascular large B-cell lymphoma (IVL). Rituximab efficacy was evaluated in Western patients with IVL, comparing outcome of 10 patients treated with rituximab + chemotherapy (R-CT) and of 20 patients treated with chem...

journal_title：British journal of haematology

authors： Ferreri AJ,Dognini GP,Bairey O,Szomor A,Montalbán C,Horvath B,Demeter J,Uziel L,Soffietti R,Seymour JF,Ambrosetti A,Willemze R,Martelli M,Rossi G,Candoni A,De Renzo A,Doglioni C,Zucca E,Cavalli F,Ponzoni M,Interna

更新日期：2008-10-01 00:00:00

abstract：:Virchow's triad describes three factors that contribute to the development of venous thrombosis: hypercoagulability, stasis and endothelial injury. Yet, extensive review of the historical literature casts doubt on the existence of a triad described by Virchow in the form it is currently quoted throughout contemporary ...

journal_title：British journal of haematology

authors： Bagot CN,Arya R

更新日期：2008-10-01 00:00:00

abstract：:Sideroblastic anaemia includes a heterogeneous group of rare conditions, characterized by decreased haem synthesis and mitochondrial iron overload, which are diagnosed by the presence of ringed sideroblasts in the bone marrow aspirate. The most frequent form is X-linked sideroblastic anaemia, caused by mutations of de...

journal_title：British journal of haematology

authors： Camaschella C

更新日期：2008-10-01 00:00:00

abstract：:A multicentre single-arm study testing the efficacy and toxicity of the oral combination of fludarabine and cyclophosphamide (FC) over 5 d in 75 patients with untreated B cell-chronic lymphocytic leukaemia. Oral FC demonstrated high efficacy with overall (OR) and complete response (CR) rates of 80% and 53%, respective...

journal_title：British journal of haematology

authors： Cazin B,Divine M,Leprêtre S,Travade P,Tournilhac O,Delmer A,Jaubert J,Feugier P,Dreyfus B,Mahé B,Grosbois B,Maloisel F,Eghbali H,Dumontet C,Bénichou J,Guibon O,Leleu X,Leporrier M,Maloum K

更新日期：2008-10-01 00:00:00

abstract：:Diamond Blackfan anaemia (DBA) is a rare, genetically and clinically heterogeneous, inherited red cell aplasia. Classical DBA affects about seven per million live births and presents during the first year of life. However, as mutated genes have been discovered in DBA, non-classical cases with less distinct phenotypes ...

journal_title：British journal of haematology

authors： Vlachos A,Ball S,Dahl N,Alter BP,Sheth S,Ramenghi U,Meerpohl J,Karlsson S,Liu JM,Leblanc T,Paley C,Kang EM,Leder EJ,Atsidaftos E,Shimamura A,Bessler M,Glader B,Lipton JM,Participants of Sixth Annual Daniella Maria Art

更新日期：2008-09-01 00:00:00

abstract：:Addition of the inflammatory cytokine interleukin (IL)-6 to the culture medium of human cord blood haematopoietic stem and progenitor cells (HSPCs) has been shown to lead to an altered stromal cell-derived factor-1alpha-dependent migratory phenotype. This study investigated whether this effect was attributed to a diff...

journal_title：British journal of haematology

authors： Kasenda B,Kassmer SH,Niggemann B,Schiermeier S,Hatzmann W,Zänker KS,Dittmar T

更新日期：2008-09-01 00:00:00

abstract：:Atrial fibrillation (AF) is a common cardiac arrhythmia with a 5-20% annual risk of stroke. Warfarin reduces this risk by at least 60%. Despite adequate anticoagulation within the target International Normalized Ratio (INR) range of 2.0-3.0, some patients still experience thrombotic and bleeding events. It is now poss...

journal_title：British journal of haematology

authors： Gatt A,van Veen JJ,Bowyer A,Woolley AM,Cooper P,Kitchen S,Makris M

更新日期：2008-09-01 00:00:00

abstract：:Transient leukaemia (TL) in neonates with Down syndrome (DS) is characterized by the transient appearance of blast cells in the peripheral blood that resolves spontaneously. Some TL patients die at an early age due to organ failure. Seventy DS patients with TL were studied retrospectively to identify clinical and labo...

journal_title：British journal of haematology

authors： Muramatsu H,Kato K,Watanabe N,Matsumoto K,Nakamura T,Horikoshi Y,Mimaya J,Suzuki C,Hayakawa M,Kojima S

更新日期：2008-08-01 00:00:00

abstract：:In agreement with a recently published manuscript, this present study demonstrated that CD38+ sub-populations had increased proliferative activity as evidenced by higher Ki-67 expression (P < 0.0001). This raised the possibility that the CD38+ fraction is exposed to an increased risk of clonal evolution. However, seri...

journal_title：British journal of haematology

authors： Lin TT,Hewamana S,Ward R,Taylor H,Payne T,Pratt G,Baird D,Fegan C,Pepper C

更新日期：2008-08-01 00:00:00

abstract：:Immunophenotypic studies, fluorescence in situ hybridization (FISH) and conventional karyotyping were used to define the clinicobiological significance of 14q32 translocations involving the immunoglobulin gene locus (14q32/IGH) in 252 chronic lymphocytic leukaemia (CLL) patients. The following regions were studied: 13...

journal_title：British journal of haematology

authors： Cavazzini F,Hernandez JA,Gozzetti A,Russo Rossi A,De Angeli C,Tiseo R,Bardi A,Tammiso E,Crupi R,Lenoci MP,Forconi F,Lauria F,Marasca R,Maffei R,Torelli G,Gonzalez M,Martin-Jimenez P,Maria Hernandez J,Rigolin GM,Cune

更新日期：2008-08-01 00:00:00

abstract：:The US National Cancer Institute's Surveillance, Epidemiology and End Results program was used to develop aetiological clues for hairy cell leukaemia (HCL). Descriptive techniques (age-adjusted incidence trends, age-specific incidence rates (IR), and age distributions-at-diagnosis) were supplemented with mathematical ...

journal_title：British journal of haematology

authors： Dores GM,Matsuno RK,Weisenburger DD,Rosenberg PS,Anderson WF

更新日期：2008-07-01 00:00:00

abstract：:Fanconi anaemia (FA) is an autosomal recessive and X-linked disease characterized by severe genetic instability and increased incidence of cancer. One explanation for this instability may be the cellular hypersensitivity to oxidative stress leading to chromosomal breaks. This study explored the possible oxidative dama...

journal_title：British journal of haematology

authors： Uziel O,Reshef H,Ravid A,Fabian I,Halperin D,Ram R,Bakhanashvili M,Nordenberg J,Lahav M

更新日期：2008-07-01 00:00:00

abstract：:We hypothesized that Wilms tumour 1 gene (WT1) expression levels in acute myeloid leukaemia (AML) patients might have predictive value and reveal molecular relapse kinetics. WT1 level was determined at diagnosis, during therapy and post-therapy follow-up in 89 patients who reached first complete remission (CR1) (952 s...

journal_title：British journal of haematology

authors： Ommen HB,Nyvold CG,Braendstrup K,Andersen BL,Ommen IB,Hasle H,Hokland P,Ostergaard M

更新日期：2008-06-01 00:00:00

abstract：:Chronic myelomonocytic leukaemia (CMML) is a clonal disorder with myelodysplastic/myeloproliferative features. Its diagnosis is based on the presence of peripheral blood monocytosis and bone marrow aspirate findings, according to World Health Organization criteria. However, bone marrow trephine biopsy (BMTB) features ...

journal_title：British journal of haematology

authors： Ngo NT,Lampert IA,Naresh KN

更新日期：2008-06-01 00:00:00

abstract：:Studies during the last three decades have enabled the development of detailed molecular insights into the structural basis of altered function in various inherited red cell membrane disorders. This review highlights our current understanding of molecular and mechanistic insights into various inherited red cell membra...

journal_title：British journal of haematology

authors： An X,Mohandas N

更新日期：2008-05-01 00:00:00

abstract：:Although the beta thalassaemia trait affects millions of people worldwide, there have been no controlled studies to determine whether it is associated with any clinical disability or abnormal physical signs. To address this question, 402 individuals were studied: 217 with beta thalassaemia trait, of whom 154 were awar...

journal_title：British journal of haematology

authors： Premawardhena A,Arambepola M,Katugaha N,Weatherall DJ

更新日期：2008-05-01 00:00:00

abstract：:Identification of the molecular basis of the beta-thalassaemias and sickle cell disease (SCD) has made it clear that patients with the same beta-globin genotypes can have very variable patterns of clinical expression. Extensive biochemical and pathophysiological studies over the last 50 years have derived two major mo...

journal_title：British journal of haematology

authors： Thein SL

更新日期：2008-05-01 00:00:00

abstract：:Idiopathic thrombotic thrombocytopenic purpura (TTP) is characterized by frequent recurrences. Effective screening for relapses will enable intervention prior to overt episodes of TTP. The present study used a modified assay to detect ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 motif, 13) ac...

journal_title：British journal of haematology

authors： Jin M,Casper TC,Cataland SR,Kennedy MS,Lin S,Li YJ,Wu HM

更新日期：2008-05-01 00:00:00

abstract：:Ph-negative chronic myeloproliferative disorders (CMPD) are characterized by constitutive Janus kinase-signal transducer and activator of transcription (JAK-STAT) activation. SOCS3, SOCS1 and PTPN6 (SHP1) are negative regulators of the JAK-STAT pathway. We investigated epigenetic and genetic inactivation of SOCS3, SOC...

journal_title：British journal of haematology

authors： Capello D,Deambrogi C,Rossi D,Lischetti T,Piranda D,Cerri M,Spina V,Rasi S,Gaidano G,Lunghi M

更新日期：2008-05-01 00:00:00

abstract：:Antibodies (Abs) that block factor VIII (FVIII) activity occur in hemophilia A patients treated with FVIII replacement therapy and severely impair treatment. In this work, we designed and synthesized ten peptides whose sequences are found in putative epitopes at the surface of a1 and C2 domains of the FVIII molecule. ...

journal_title：British journal of haematology

authors： Chaves DG,Velloso-Rodrigues C,Moreau V,Nguyen C,Villard S,Belisário AR,Granier C,Santoro MM

更新日期：2008-05-01 00:00:00

abstract：:Changes in spleen size postallogeneic haematopoietic stem cell transplantation (HSCT) in patients with primary myelofibrosis have been poorly characterized. We analysed 10 patients with myelofibrosis and splenomegaly following a reduced-intensity allogeneic HSCT. All patients fully engrafted donor cells including five...

journal_title：British journal of haematology

authors： Ciurea SO,Sadegi B,Wilbur A,Alagiozian-Angelova V,Gaitonde S,Dobogai LC,Akard LP,Hoffman R,Rondelli D

更新日期：2008-04-01 00:00:00