Constitutional trisomy 8p11.21-q11.21 mosaicism: a germline alteration predisposing to myeloid leukaemia.

abstract：:Myeloperoxidase (MPO) catalyses the formation of hypochlorous acid and is involved in many (patho)physiological processes. The present study was designed to determine the effect of two MPO promoter polymorphisms (463G/A and 129G/A) on enzyme activity. In 243 healthy controls, genotypes were determined and MPO activity...

journal_title：British journal of haematology

authors： Rutgers A,Heeringa P,Giesen JE,Theunissen RT,Jacobs H,Tervaert JW

更新日期：2003-11-01 00:00:00

abstract：:This is an historical account of the randomised trials in chronic lymphocytic leukaemia in the UK between the years 1978 to 2004, describing their gestation, the treatments used and the main lessons learnt. Those lessons include: (1) how best to use chlorambucil, which was the first effective treatment for CLL; (2) th...

journal_title：British journal of haematology

authors： Catovsky D,Else M

更新日期：2020-11-01 00:00:00

abstract：:To assess and define the effects of heterocellular hereditary persistence of fetal haemoglobin (HPFH) on the haematological phenotype of heterozygous beta-thalassaemia, we have studied a large kindred that included a total of 204 subjects with 60 beta-thalassaemia carriers, of whom 35 were also heterozygous, and five ...

journal_title：British journal of haematology

authors： Garner C,Dew TK,Sherwood R,Rees D,Thein SL

更新日期：2003-10-01 00:00:00

abstract：:Two new glucose-6-phosphate dehydrogenase (G6PD, D-glucose 6-phosphate: NADP oxido reductase, E.C. 1.1.1.49) variants, designated G6PD Napoli and G6PD Ferrara II, are described in propositi from two unrelated families. Characterization side by side of the two variants according to W.H.O. recommendations reveals minor ...

journal_title：British journal of haematology

authors： De Flora A,Morelli A,Benatti U,Giuntini P,Ferraris AM,Galiano S,Ravazzolo R,Gaetani GF

更新日期：1981-07-01 00:00:00

abstract：:Myelofibrosis is characterized by splenomegaly and debilitating constitutional symptoms that negatively impact patients' quality of life. ROBUST, a UK, open-label, phase II study, evaluated the safety and efficacy of ruxolitinib in patients with myelofibrosis (N = 48), including intermediate-1 risk patients. The prima...

journal_title：British journal of haematology

authors： Mead AJ,Milojkovic D,Knapper S,Garg M,Chacko J,Farquharson M,Yin J,Ali S,Clark RE,Andrews C,Dawson MK,Harrison C

更新日期：2015-07-01 00:00:00

abstract：:Peripheral blood globin synthesis studies were done in 11 patients with delta beta-thalassaemia trait, Hb S-delta beta-thalassaemia or delta beta/betao-thalassaemia from two black and two Caucasian families. All patients had elevated Hb F and normal or decreased Hb A2 levels and 10 had family studies confirming the di...

journal_title：British journal of haematology

authors： Kinney TR,Friedman S,Cifuentes E,Kim HC,Schwartz E

更新日期：1978-01-01 00:00:00

abstract：:The replicative potential of human CD56(+) CD3(-) natural killer (NK) cells is unknown. We found that by exposing NK cells to the leukaemic cell line K562 genetically modified to express 4-1BB ligand and interleukin 15 (K562-mb15-41BBL), they expanded for up to 30 population doublings, achieving numbers that ranged fr...

journal_title：British journal of haematology

authors： Fujisaki H,Kakuda H,Imai C,Mullighan CG,Campana D

更新日期：2009-06-01 00:00:00

abstract：:Patients with sickle cell disease (SCD) with high fetal haemoglobin (HbF) tend to have a lower incidence of complications and longer survival due to inhibition of deoxyhaemoglobin S (HbS) polymerisation by HbF. HbF-containing cells, namely F cells, are strongly influenced by genetic factors. We measured the percentage...

journal_title：British journal of haematology

authors： Urio F,Nkya S,Rooks H,Mgaya JA,Masamu U,Zozimus Sangeda R,Mmbando BP,Brumat M,Mselle T,Menzel S,Luzzatto L,Makani J

更新日期：2020-10-19 00:00:00

abstract：:The role of autologous stem cell transplantation (ASCT) and the optimal timing of such transplants in patients with follicular lymphoma (FL) remains contentious. We present a single-centre experience documenting the outcomes of 70 FL patients who underwent BEAM (carmustine, cytarabine, etopside, melphalan)-conditioned...

journal_title：British journal of haematology

authors： Kothari J,Peggs KS,Bird A,Thomson KJ,Morris E,Virchis AE,Lambert J,Goldstone AH,Linch DC,Ardeshna KM

更新日期：2014-05-01 00:00:00

abstract：:Heparin-induced thrombocytopenia (HIT) is mediated by a heparin-dependent antibody/platelet factor 4/heparin complex binding to platelets via the Fc gamma receptor (type IIA). A single base polymorphism at position 131 of Fc gamma RIIA changes the native arginine to histidine. In the presence of murine monoclonal IgG1...

journal_title：British journal of haematology

authors： Burgess JK,Lindeman R,Chesterman CN,Chong BH

更新日期：1995-11-01 00:00:00

abstract：:Patients with idiopathic anaemias associated with abnormal sideroblasts were defined according to morphologic and ferrokinetic criteria and the haematologic and biochemical effects of vitamin B6 therapy were evaluated. While all patients presented similar clinical pictures, peripheral blood changes and bone marrow abn...

journal_title：British journal of haematology

authors： Solomon LR,Hillman RS

更新日期：1979-06-01 00:00:00

abstract：:Very Late Antigen-4 (VLA-4, α4β1-integrin, ITGA4) orchestrates cell-cell and cell-endothelium adhesion. Given the proposed role of VLA-4 in sickle cell disease (SCD) pathophysiology, we evaluated the ability of the VLA-4 blocking antibody natalizumab to inhibit SCD blood cell adhesion. Natalizumab recognized surface V...

journal_title：British journal of haematology

authors： White J,Krishnamoorthy S,Gupta D,Lancelot M,Moore N,Sarnaik S,Hobbs WE 2nd,Light DR,Hines P

更新日期：2016-09-01 00:00:00

abstract：:In a prospective phase II trial, pentostatin combined with cyclophosphamide and rituximab (PCR) induced strong responses and was well-tolerated in previously untreated patients with advanced-stage, indolent non-Hodgkin lymphoma (iNHL). After a median patient follow-up of more than 108 months, we performed an intent-to...

journal_title：British journal of haematology

authors： Khashab T,Hagemeister F,Romaguera JE,Fanale MA,Pro B,McLaughlin P,Rodriguez MA,Neelapu SS,Fayad L,Younes A,Feng L,Vega F,Kwak LW,Samaniego F

更新日期：2019-05-01 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：:The feasibility of marrow cryopreservation for autologous bone marrow transplantation after 7 d in liquid culture was assessed in 10 leukaemic patients. A median of 0.17 x 10(8) nucleated cells/kg and 0.4 x 10(4) CFU-GM/kg could be collected after the complete procedure, with overall a consistent cell loss. Long-term ...

journal_title：British journal of haematology

authors： Stoppa AM,Maraninchi D,Lafage M,Mannoni P,Novakovitch G,Blaise D,Gaspard MH,Michel G,Carcassonne Y

更新日期：1989-08-01 00:00:00

abstract：:During treatment of human red cells with phospholipase A2 from bee venom, a linear increase of the MCV and of the osmotic fragility occurs in parallel with the cleavage of the accessible phospholipids. However, even after maximal hydrolysis, i.e. degradation of up to 65% of the phosphatidylcholines and up to 6% of the...

journal_title：British journal of haematology

authors： Vaysse J,Pilardeau P,Gattegno L,Garnier M

更新日期：1987-03-01 00:00:00

abstract：:Neutrophils are amongst the first immune cells to arrive at sites of infection and play an important role as the host's first line of defence against invading pathogens. Defects of neutrophil number or function are usually recognized clinically by recurrent infections that often are life-threatening. Over the last few...

journal_title：British journal of haematology

authors： Bouma G,Ancliff PJ,Thrasher AJ,Burns SO

更新日期：2010-11-01 00:00:00

abstract：:Bone marrow culture in a patient with aplastic anaemia responding to anabolic steroid (methandienone) therapy, showed an unusually high degree of growth in unstimulated cultures. Growth in unstimulated cultures is due to factors with colony stimulating activity (CSA) released by monocyte macrophages in the bone marrow...

journal_title：British journal of haematology

authors： Francis GE,Berney JJ,Bateman SM,Hoffbrand AV

更新日期：1977-08-01 00:00:00

abstract：:2,3-Dihydroxybenzoic acid (2,3-DHB) inhibits the second wave of platelet aggregation release of serotonin, and malonaldehyde production. The effect is concentration dependent and reversible in vitro and in vivo. 50% inhibition of platelet aggregation induced in vitro with 70 micron adrenaline was obtained with 1.5 mM ...

journal_title：British journal of haematology

authors： Greenberg M,Grady RW,Peterson CM

更新日期：1977-12-01 00:00:00

abstract：:Epstein-Barr virus-associated haemophagocytic lymphohistiocytosis (EBV-HLH) is one of the most common subtypes of secondary HLH. However, more than 30% of patients do not respond to traditional treatment. Here, we investigated the efficacy and safety of the L-DEP regimen as a salvage therapy for paediatric refractory ...

journal_title：British journal of haematology

authors： Zhao Y,Li Z,Zhang L,Lian H,Ma H,Wang D,Zhao X,Zhang Q,Wang T,Zhang R

更新日期：2020-11-01 00:00:00

abstract：:The Fanconi anaemia protein FANCD2 suppresses PPARƔ to maintain haematopoietic stem cell's (HSC) function; however, the underlying mechanism is not known. Here we show that FANCD2 acts in concert with the Notch target HES1 to suppress inflammation-induced PPARƔ in HSC maintenance. Loss of HES1 exacerbates FANCD2-KO HS...

journal_title：British journal of haematology

authors： Wu L,Li X,Lin Q,Chowdhury F,Mazumder MH,Du W

更新日期：2020-11-22 00:00:00

abstract：:Deregulated Hedgehog (Hh) signalling activity may be associated with a broad range of cancer types and hence has become an attractive target for therapeutic intervention. Although initial haematological interest focused on the therapeutic targeting of this pathway in chronic myeloid leukaemia), small molecule inhibito...

journal_title：British journal of haematology

authors： Khan AA,Harrison CN,McLornan DP

更新日期：2015-08-01 00:00:00

abstract：:We studied the activity and stage of chronic liver disease in 45 HCV-seropositive/HIV-seronegative patients with severe haemophilia followed for at least 10 years. HCV-RNA was detected in serum in 36 patients (80%) Viraemic cases were further analysed for HCV genotypes: 10 (28%) were infected by type 1a, 10 (28%) by t...

journal_title：British journal of haematology

authors： Tagariello G,Pontisso P,Davoli PG,Ruvoletto MG,Traldi A,Alberti A

更新日期：1995-11-01 00:00:00

abstract：:High-dose cytosine arabinoside (AraC)-containing regimens have shown the highest antileukaemic efficacy of all currently used regimens in the treatment of acute myeloid leukaemia (AML). This study aimed at increasing the antileukaemic potential of high-dose AraC by raising intracellular levels of AraC triphosphate (Ar...

journal_title：British journal of haematology

authors： Braess J,Wegendt C,Jahns-Streubel G,Kern W,Keye S,Unterhalt M,Schleyer E,Hiddemann W

更新日期：2000-05-01 00:00:00

abstract：:Clinical and cytogenetic data were analysed in 54 patients with acute non-lymphocytic leukaemias (ANLL) or MDS (myelodysplastic syndromes) and deletion of the long arm of chromosome 7 (7q-), in order to determine if there is a commonly deleted region in 7q and to establish possible correlations between karyotypic feat...

journal_title：British journal of haematology

authors： Velloso ER,Michaux L,Ferrant A,Hernandez JM,Meeus P,Dierlamm J,Criel A,Louwagie A,Verhoef G,Boogaerts M,Michaux JL,Bosly A,Mecucci C,Van den Berghe H

更新日期：1996-03-01 00:00:00

abstract：:A chromogenic factor Xa generation method has been developed for comparing the co-factor activity of factor VIII concentrates at physiological factor VIII concentrations (1 iu/ml). In the presence of thrombin all concentrates gave similar rapid rates of factor Xa generation, but in the absence of thrombin there were m...

journal_title：British journal of haematology

authors： Kemball-Cook G,Tubbs JE,Dawson NJ,Barrowcliffe TW

更新日期：1993-06-01 00:00:00

abstract：:In HbSC disease, as in sickle cell anaemia, there is a spectrum of clinical severity. A reduced mean corpuscular volume and haemoglobin concentration, traits typical of thalassaemia, might retard sickling. We therefore ascertained the prevalence of alpha-thalassaemia in 53 adults with HbSC disease and related alpha-gl...

journal_title：British journal of haematology

authors： Steinberg MH,Coleman MB,Adams JG,Platica O,Gillette P,Rieder RF

更新日期：1983-11-01 00:00:00

abstract：:Eradication of Helicobacter pylori infection has been associated with the correction of thrombocytopenia in patients with idiopathic thrombocytopenic purpura (ITP). We have analysed the response to eradication of H. pylori in a series of 56 adult patients with chronic ITP. Forty patients had H. pylori infection (71%) ...

journal_title：British journal of haematology

authors： Jarque I,Andreu R,Llopis I,De la Rubia J,Gomis F,Senent L,Jiménez C,Martín G,Martínez JA,Sanz GF,Ponce J,Sanz MA

更新日期：2001-12-01 00:00:00

abstract：:When monocytic leukaemia line U937 cells were incubated in the presence of HgCl2 there was a rapid increase in tissue factor (TF)-dependent procoagulant activity, reaching a maximum (equivalent to the total TF activity observed when cells had been subjected to a freeze/thaw cycle) after 15 min at 50 microM HgCl2 and a...

journal_title：British journal of haematology

authors： Kaneko H,Kakkar VV,Scully MF

更新日期：1994-05-01 00:00:00

abstract：:Neutrophil hypersegmentation (NH) is an important haematological feature of cobalamin or folate deficiency. As iron deficiency and folate deficiency often occur in the same target groups it is important to establish whether iron deficiency alone is a cause of NH. We report a case-control study which addresses this iss...

journal_title：British journal of haematology

authors： Westerman DA,Evans D,Metz J

更新日期：1999-12-01 00:00:00