A new Turkish type of beta-thalassaemia major with homozygosity for two non-consecutive 7.6 kb deletions of the psi beta and beta genes and an intact delta gene.

abstract：:We describe and compare five assay systems for Protein C (PC) in human plasma; a functional assay for PC activity, Laurell electroimmunoassay with EDTA or calcium (EDTA-Laurell or Ca-Laurell), radioimmunoassay (RIA) and immunoradiometric assay (IRMA). The lower limit of sensitivity of PC in normal reference plasma was...

journal_title：British journal of haematology

authors： Mikami S,Tuddenham EG

更新日期：1986-01-01 00:00:00

abstract：:This retrospective study aimed to analyse the impact on overall survival (OS) and event-free survival (EFS) of chimaerism status and kinetics following allogeneic conventional and reduced-intensity conditioning haematopoietic stem cell transplantation, and to compare this with the impact of other well-known factors. W...

journal_title：British journal of haematology

authors： Michallet AS,Fürst S,Le QH,Dubois V,Praire A,Nicolini F,Thomas X,Rafii H,Gebuhrer L,Michallet M

更新日期：2005-03-01 00:00:00

abstract：:Acute graft-versus-host disease (GvHD) is an important complication of bone marrow transplantation in humans. Risk factors are imprecisely defined and controversial. We analysed data from 2036 recipients of HLA-identical sibling transplants for leukaemia or aplastic anaemia to identify risk factors for GvHD. Analyses ...

journal_title：British journal of haematology

authors： Gale RP,Bortin MM,van Bekkum DW,Biggs JC,Dicke KA,Gluckman E,Good RA,Hoffmann RG,Kay HE,Kersey JH

更新日期：1987-12-01 00:00:00

abstract：:Chronic myeloid leukaemia invariably progresses from a drug-sensitive to a drug-resistant, aggressive acute leukaemia. The mechanisms responsible for this are unknown, although loss of p53 has been reported in approximately 25% of cases. Elevated expression of Bcr-Abl is also associated with disease progression. We ha...

journal_title：British journal of haematology

authors： Keeshan K,Cotter TG,McKenna SL

更新日期：2003-10-01 00:00:00

abstract：:High-dose chemotherapy with autologous stem cell transplantation (SCT) is widely used as a treatment for systemic AL amyloidosis, but its efficacy has not been proved and it has substantial toxicity in this setting. We report here the outcome of 92 patients evaluated at the UK National Amyloidosis Centre who underwent...

journal_title：British journal of haematology

authors： Goodman HJ,Gillmore JD,Lachmann HJ,Wechalekar AD,Bradwell AR,Hawkins PN

更新日期：2006-08-01 00:00:00

abstract：:Data on endogenous thrombopoietin (TPO) levels and their regulation in myelodysplastic syndromes (MDS) are sparse. We examined the plasma TPO level of 85 MDS patients by a sensitive enzyme immunoassay and the platelet expression of TPO receptor (TPO-R) protein, which metabolizes endogenous TPO, in 19 MDS patients with...

journal_title：British journal of haematology

authors： Tamura H,Ogata K,Luo S,Nakamura K,Yokose N,Dan K,Tohyama K,Yoshida Y,Hamaguchi H,Sakamaki H,Kuwaki T,Tahara T,Kato T,Nomura T

更新日期：1998-12-01 00:00:00

abstract：:Glanzmann's thrombasthenia (GT) results from a qualitative or quantitative defect of GPIIb-IIIa complexes (integrin alphaIIbbeta3). the fibrinogen receptor on platelets. This integrin plays a critical role in platelet aggregation. In this report we describe the molecular abnormalities of a patient with clinical and la...

journal_title：British journal of haematology

authors： Ruan J,Peyruchaud O,Alberio L,Valles G,Clemetson K,Bourre F,Nurden AT

更新日期：1998-09-01 00:00:00

abstract：:Leucocyte alkaline phosphatase (LAP) is a marker of post-mitotic granulocytes and its activity is reduced or absent in chronic myelogenous leukaemia (CML) granulocytes as a consequence of LAP messenger RNA (mRNA) deficiency. We provide evidence that along the granulocytic maturation in normal marrow, the acquisition o...

journal_title：British journal of haematology

authors： Dotti G,Garattini E,Borleri G,Masuhara K,Spinelli O,Barbui T,Rambaldi A

更新日期：1999-04-01 00:00:00

abstract：:The platelets of a young man with the grey platelet syndrome were severely depleted of all seven alpha-granule proteins assayed as well as partially deficient in alpha-mannosidase and alpha-fucosidase; four other lysosomal enzymes were present in normal concentrations. Total platelet 5-hydroxytryptamine (5HT) and aden...

journal_title：British journal of haematology

authors： Srivastava PC,Powling MJ,Nokes TJ,Patrick AD,Dawes J,Hardisty RM

更新日期：1987-04-01 00:00:00

abstract：:We have previously shown that allogeneic bone marrow transplantation (BMT) with cryopreserved donor marrow cells can be used without prolonging the engraftment time or interfering with the reconstitution of haemopoiesis. In this report we extend our initial observations of the first 40 patients who underwent allogenei...

journal_title：British journal of haematology

authors： Stockschläder M,Hassan HT,Krog C,Krüger W,Löliger C,Horstman M,Altnöder M,Clausen J,Grimm J,Kabisch H,Zander A

更新日期：1997-02-01 00:00:00

abstract：:Drug-induced agranulocytosis (neutrophil count <0.5 x 10(9)/l) is a rare haematological complication with an incidence of no more than 10 cases per million inhabitants per year in Europe. Over the past few years there has been a steady decline in mortality rate, (currently at <5%), which can be partly explained by ear...

journal_title：British journal of haematology

authors： Andrès E,Maloisel F,Zimmer J

更新日期：2010-07-01 00:00:00

abstract：:In acute lymphoblastic leukaemia (ALL), investigation of minimal residual disease by conventional morphology and immunology fails to detect levels of residual disease of < 1 leukaemic in 10-100 normal cells. The use of polymerase chain reaction (PCR) to exploit the diversity of the complementarity determining region (...

journal_title：British journal of haematology

authors： Chim JC,Coyle LA,Yaxley JC,Cole-Sinclair MF,Cannell PK,Hoffbrand VA,Foroni L

更新日期：1996-01-01 00:00:00

abstract：:The Dutch beta zero-thalassaemia has few clinical symptoms in homozygotes, elevated fetal haemoglobin (4-11%) in heterozygotes, and has a DNA deletion previously estimated as 10 kb which removes the beta-globin gene (Gilman et al, 1984). A DNA fragment containing the breakpoints of the Dutch beta zero-thalassaemia del...

journal_title：British journal of haematology

authors： Gilman JG

更新日期：1987-11-01 00:00:00

abstract：:Severe neutropenia and protracted thrombocytopenia remain serious clinical problems following cord blood transplantation (CBT) due to the paucity of stem and progenitor cells in the grafts. Administration of ex-vivo expanded megakaryocyte progenitor cells may facilitate platelet production. We propose a novel strategy...

journal_title：British journal of haematology

authors： Deutsch V,Hubel E,Kay S,Ohayon T,Katz BZ,Many A,Zander A,Naparstek E,Grisaru D

更新日期：2010-04-01 00:00:00

abstract：:Dendritic cell (DC) differentiation was investigated in samples from two acute promyelocytic leukaemia (APL) patients with classic translocation t(15;17)(q22;q21). After 18 d of culture in the presence of granulocyte-macrophage colony-stimulating factor, interleukin 4 and tumour necrosis factor alpha, 10-15% of pathol...

journal_title：British journal of haematology

authors： Rigolin GM,Della Porta M,Bigoni R,Tieghi A,Cuneo A,Castoldi G

更新日期：2001-09-01 00:00:00

abstract：:Karyotypic studies of bone marrow were conducted in 79 previously untreated patients with multiple myeloma who received a standard programme of chemotherapy. An abnormal karyotype was observed in 46% of patients, virtually all showing multiple abnormalities consistent with a long period of preclinical clonal evolution...

journal_title：British journal of haematology

authors： Seong C,Delasalle K,Hayes K,Weber D,Dimopoulos M,Swantkowski J,Huh Y,Glassman A,Champlin R,Alexanian R

更新日期：1998-04-01 00:00:00

abstract：:Until recently, primary central nervous system lymphoma (PCNSL) was associated with a uniformly dismal prognosis. It is now reasonable to anticipate long-term survival and possibly cure for a significant proportion of patients diagnosed with PCNSL. Accumulated data generated over the past 10 years has provided evidenc...

journal_title：British journal of haematology

authors： Wang CC,Carnevale J,Rubenstein JL

更新日期：2014-08-01 00:00:00

abstract：:Liposomes prepared from rabbit brain extracts (RBE) and individual pure lipids (high phosphatidyl serine content, HIPS) were compared with frozen-thawed platelets (PLTS) in the dilute Russell Viper venom time (dRVVt). While all three preparations demonstrated sensitivity to lupus anticoagulant (LA) the highest detecti...

journal_title：British journal of haematology

authors： Stevenson KJ,Seddon JM

更新日期：1994-03-01 00:00:00

abstract：:Continuation chemotherapy is a key component of the treatment of childhood acute lymphoblastic leukaemia. During this treatment phase, weekly dose adjustments are carried out based on current and historical full blood counts (FBCs). The dose decision pathway is complex and suboptimal therapy may result if information ...

journal_title：British journal of haematology

authors： Bury J,Hurt C,Roy A,Cheesman L,Bradburn M,Cross S,Fox J,Saha V

更新日期：2005-06-01 00:00:00

abstract：:Minimal residual disease (MRD) assessments were performed retrospectively after allogeneic stem cell transplantation (SCT) in 32 patients (23 children and nine adults) with acute lymphoblastic leukaemia (ALL). Using immunoglobulin and T-cell receptor rearrangements as clonal markers, MRD was detected after SCT in nine...

journal_title：British journal of haematology

authors： Uzunel M,Jaksch M,Mattsson J,Ringdén O

更新日期：2003-09-01 00:00:00

abstract：:Platelet and cell stimulation lead to plasma membrane remodelling, resulting in phosphatidylserine (PS) externalisation in the outer leaflet of the membrane, associated with the shedding of PS-rich microparticles (MPs), and contributes to thrombin generation by promoting the assembly of coagulation enzyme complexes. A...

journal_title：British journal of haematology

authors： Proulle V,Hugel B,Guillet B,Grunebaum L,Lambert T,Freyssinet JM,Dreyfus M

更新日期：2005-11-01 00:00:00

abstract：:Reversible aggregation of red blood cells (RBC) plays an important role in determining blood flow properties, and it is this aggregation which increases blood viscosity at low shear rates. The structure and sites of venous thrombi, as well as the fact that stasis is a major predisposing factor in venous thrombosis, su...

journal_title：British journal of haematology

authors： Chabanel A,Horellou MH,Conard J,Samama MM

更新日期：1994-09-01 00:00:00

abstract：:A patient with apparent anaemia and thrombocytopenia caused by a monoclonal paraprotein is described. The patient's serum contained a monoclonal IgM kappa, a cryoglobulin and a cold agglutinin. The cryoglobulin, similar to the serum paraprotein, was a monoclonal IgM kappa. Serum was studied to determine the relationsh...

journal_title：British journal of haematology

authors： Pujol M,Ribera JM,Jimenez C,Ribera A,Abad E,Feliu E

更新日期：1998-03-01 00:00:00

abstract：:Human amniotic fluid has been shown to contain blood group i as well as I antigens. Crude extracts of amniotic fluids at 16-23 weeks of gestation were in general more active than those obtained at term. A pool of amniotic fluids which had A, B, H as well as I and i activity was fractionated with an insolubilized anti-...

journal_title：British journal of haematology

authors： Feizi T,Cederqvist LL,Childs R

更新日期：1975-08-01 00:00:00

abstract：:This work aimed to provide a means of assaying directly the effects of transient expression of introduced genes on the survival, proliferation, lineage commitment and differentiation of haemopoietic progenitor cells. For this purpose, we have developed a system that allows isolation of productively transfected, muliti...

journal_title：British journal of haematology

authors： McIvor ZJ,Heyworth CM,Johnson BA,Pearson S,Fiegler H,Hampson L,Dexter TM,Cross MA

更新日期：2000-09-01 00:00:00

abstract：:Endogenous production of carbon monoxide (VCO), red cell survival and iron kinetics were studied in 15 subjects with Hodgkin's disease. The subjects were divided into two groups, namely: eight patients with anaemia (group A, haemoglobin (Hb) concentration less than 11.5 g/dl) and seven patients without anaemia (group ...

journal_title：British journal of haematology

authors： Cavallin-Ståhl E,Mercke C,Lundh B

更新日期：1976-02-01 00:00:00

abstract：:In recent years there has been a paradigm shift in our understanding of the epidemiology and clinical features of hepatitis E virus (HEV) infection. Once classically described as an acute hepatitis associated with waterborne outbreaks in areas of poor sanitation, HEV is now recognised to be endemic in Europe and is pr...

journal_title：British journal of haematology

authors： O'Gorman J,Burke Á,O'Flaherty N

更新日期：2018-06-01 00:00:00

abstract：:The impact of age upon therapeutic response to unfractionated heparin (UFH) in children is proposed to reflect quantitative and potentially qualitative differences in coagulation proteins across childhood. This study explores the UFH-dependent tissue factor pathway inhibitor (TFPI) release in children compared to prev...

journal_title：British journal of haematology

authors： Newall F,Johnston L,Summerhayes R,Lane G,Cranswick N,Monagle P,Ignjatovic V

更新日期：2010-11-01 00:00:00

abstract：:A new alpha thalassemia defect has been detected in the South African population. Restriction mapping of the alpha globin gene cluster in affected individuals has established that the defect is associated with the removal of 22.8-23.7 kb of DNA, including the psi zeta 1, psi alpha 1, psi alpha 2, alpha 2 and alpha 1 g...

journal_title：British journal of haematology

authors： Vandenplas S,Higgs DR,Nicholls RD,Bester AJ,Mathew CG

更新日期：1987-08-01 00:00:00

abstract：:Tissue factor pathway inhibitor (TFPI) is released to circulating blood after intravenous (i.v.) and subcutaneous (s.c.) injections of heparins, and may thus contribute to the antithrombotic effect of heparins. We have recently shown that total TFPI activity, plasma free TFPI antigen, and heparin releasable TFPI were ...

journal_title：British journal of haematology

authors： Bendz B,Hansen JB,Andersen TO,Ostergaard P,Sandset PM

更新日期：1999-12-01 00:00:00