Platelet von Willebrand factor--structure, function and biological importance.

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：:Five out of nine adults (55%) with lymphoblastic disease developed severe avascular necrosis of bone (AVN) when treated with a Berlin-Frankfurt-Munster (BFM) ALL protocol similar to the current joint MRC-ECOG ALL trial (UKALL XII). The principal purpose of these intensified regimens is to improve long-term disease-fre...

journal_title：British journal of haematology

authors： Chan-Lam D,Prentice AG,Copplestone JA,Weston M,Williams M,Hutton CW

更新日期：1994-01-01 00:00:00

abstract：:In four unrelated patients with chronic haemolysis and markedly reduced red blood cell (RBC) glutathione (49.5%, 12.6%, 11.5% and 15% of the normal concentration respectively), a severe glutathione synthetase (GSH-S, EC 6.3.2.3) deficiency was found. One case exhibited a neonatal haemolytic anaemia associated with oxo...

journal_title：British journal of haematology

authors： Corrons JL,Alvarez R,Pujades A,Zarza R,Oliva E,Lasheras G,Callis M,Ribes A,Gelbart T,Beutler E

更新日期：2001-02-01 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：:Further analysis of rat urine containing labelled catabolites derived from administered 3H- or 14C-labelled folic acid is described. The results support previously described studies, and show that folate catabolism in the rat takes place by cleavage of the C9-N10 bond, and not by excretion of inactive forms of the vit...

journal_title：British journal of haematology

authors： Murphy M,Boyle PH,Weir DG,Scott JM

更新日期：1978-02-01 00:00:00

abstract：:Erythroid regeneration is an important and separate element in the engraftment process in allogeneic and autologous bone marrow transplantation (alloBMT, autoBMT). Qualitative visual reticulocyte counting has proved inadequate in the evaluation of erythropoiesis after BMT but automated flow cytometry now allows the re...

journal_title：British journal of haematology

authors： Davies SV,Cavill I,Bentley N,Fegan CD,Poynton CH,Whittaker JA

更新日期：1992-05-01 00:00:00

abstract：:The number of granulocyte-macrophage clones formed in agar culture of bone marrow is dependent on levels of colony stimulating activity (CSA) a proposed in vivo haemopoietic regulator. A dose-response relationship for stimulation of human haemopoietic cells by CSA is demonstrated, which could be explained by threshold...

journal_title：British journal of haematology

authors： Francis GE,Berney JJ,Hoffebrand AV

更新日期：1977-04-01 00:00:00

abstract：:Primary mediastinal B-cell lymphoma (PMBL) shows chromosome 9p anomalies in 50% of cases. Based on reports that p16INK4A gene, located on this chromosomal arm, is frequently altered in aggressive lymphomas, we analysed for alterations of this gene in 27 cases of PMBL, which were part of a series of 32 PMBL cases that ...

journal_title：British journal of haematology

authors： Scarpa A,Moore PS,Rigaud G,Inghirami G,Montresor M,Menegazzi M,Todeschini G,Menestrina F

更新日期：1999-10-01 00:00:00

abstract：:We have investigated the potency of the C677T mutation in the methylene tetrahydrofolate reductase (MTHFR) gene as a genetic risk factor in women with a history of early (12 weeks gestation) recurrent miscarriage (three or more consecutive pregnancy losses). Fifty-seven of the tota...

journal_title：British journal of haematology

authors： Holmes ZR,Regan L,Chilcott I,Cohen H

更新日期：1999-04-01 00:00:00

abstract：:To resolve the controversy concerning the role of p53 in the killing of resting lymphocytes by purine nucleoside analogues, we examined the cytotoxic effects of chlorodeoxyadenosine, fludarabine and deoxycoformycin (plus deoxyadenosine) on unstimulated spleen cells from p53-knockout versus wild-type mice. p53-knockout...

journal_title：British journal of haematology

authors： Pettitt AR,Clarke AR,Cawley JC,Griffiths SD

更新日期：1999-06-01 00:00:00

abstract：:We evaluated the blood from 150 patients with primary AL-amyloidosis for circulating monoclonal plasma cells using a sensitive slide-based immunofluorescence technique. The percentage of monoclonal blood plasma cells (BPC) that were in S-phase was determined by the bromodeoxyuridine labelling index (BLI). Monoclonal B...

journal_title：British journal of haematology

authors： McElroy EA Jr,Witzig TE,Gertz MA,Greipp PR,Kyle RA

更新日期：1998-02-01 00:00:00

abstract：:We have assessed the effects of the diphosphonate, dichloromethylene diphosphonate (Cl2MDP), in 19 patients with hypercalcaemia and increased bone resorption due to myeloma. Cl2MDP (800-3200 mg daily by mouth or 300 mg daily by intravenous infusion) decreased plasma calcium and biochemical indices of increased bone re...

journal_title：British journal of haematology

authors： Paterson AD,Kanis JA,Cameron EC,Douglas DL,Beard DJ,Preston FE,Russell RG

更新日期：1983-05-01 00:00:00

abstract：:We have investigated the molecular basis of alpha thalassaemia in the so-called 'Cape Coloured' population of Cape Town. DNA from 17 cases was analysed by Southern blotting and hybridization with an alpha globin complementary DNA probe. Three types of alpha thalassaemia genetic determinants were detected: the 3.5 kb d...

journal_title：British journal of haematology

authors： Mathew CG,Rousseau J,Rees JS,Harley EH

更新日期：1983-09-01 00:00:00

abstract：:Genome-wide association studies (GWAS) have shown that the 8q24 region harbours multiple independent cancer susceptibility loci, even though it is devoid of genes. Given that no GWAS data are currently available for multiple myeloma (MM), we tested the hypothesis that genetic variants in this region could play a role ...

journal_title：British journal of haematology

authors： Campa D,Martino A,Sainz J,Buda G,Jamroziak K,Weinhold N,Vieira Reis RM,García-Sanz R,Jurado M,Ríos R,Szemraj-Rogucka Z,Marques H,Lesueur F,Bugert P,Moreno V,Szemraj J,Orciuolo E,Gemignani F,Rossi AM,Dumontet C,Pet

更新日期：2012-05-01 00:00:00

abstract：:Patients with aplastic anaemia (AA) frequently develop paroxysmal nocturnal haemoglobinuria (PNH) as a late complication. We investigated the frequency of the development of PNH features including a glycosyl phosphatidylinositol (GPI) anchoring defect in 73 Japanese patients with AA. A deficient expression of CD59 was...

journal_title：British journal of haematology

authors： Azenishi Y,Ueda E,Machii T,Nishimura J,Hirota T,Shibano M,Nakao S,Kinoshita T,Mizoguchi H,Kitani T

更新日期：1999-03-01 00:00:00

abstract：:Imatinib mesylate is useful for facilitating allogeneic stem cell transplantation (allo-SCT) in advanced-phase chronic myelogenous leukaemia (AP-CML) patients. However, although the side-effects of imatinib are usually minor, cardiac morbidity can develop as a latent adverse effect post SCT when a myeloablative SCT is...

journal_title：British journal of haematology

authors： Sohn SK,Kim JG,Kim DH,Lee KB

更新日期：2003-05-01 00:00:00

abstract：:We assessed the clinical utility of next-generation sequencing (NGS)-based monitoring of minimal residual disease (MRD) in a uniformly treated cohort of 79 patients with paediatric B-cell acute lymphoblastic leukaemia. Bone marrow samples were collected at the time of diagnosis, days 33 and 80, pre- (4-5 months) and p...

journal_title：British journal of haematology

authors： Sekiya Y,Xu Y,Muramatsu H,Okuno Y,Narita A,Suzuki K,Wang X,Kawashima N,Sakaguchi H,Yoshida N,Hama A,Takahashi Y,Kato K,Kojima S

更新日期：2017-01-01 00:00:00

abstract：:Low concentrations of collagen and anticardiolipin antibodies (ACLA), which were raised in rabbits by immunization with cardiolipin (CL), co-operatively activated human gel-filtrated platelets (GFP). GFP activated by adding ACLA 5 min prior to collagen (ACLA + Col) showed strong responses in cytosolic Ca2+ mobilizatio...

journal_title：British journal of haematology

authors： Wang L,Su CY,Chou KY,Wang CT

更新日期：2002-09-01 00:00:00

abstract：:During periods of immunosuppression, such as postallogeneic stem cell transplantation (SCT), patients are at significant risk for severe viral infections. Human adenovirus (HAdV) infection is a serious complication post-SCT, especially in children. Virus-specific T cells are essential for the clearance of HAdV, as ant...

journal_title：British journal of haematology

authors： Feuchtinger T,Matthes-Martin S,Richard C,Lion T,Fuhrer M,Hamprecht K,Handgretinger R,Peters C,Schuster FR,Beck R,Schumm M,Lotfi R,Jahn G,Lang P

更新日期：2006-07-01 00:00:00

abstract：:The F5 G1691A (Factor V Leiden) and F2 G20210A (prothrombin) mutations are linked to an increase in the incidence rate of venous thromboembolism (VTE), but their effects are highly variable. We investigated whether the effects of smoking and obesity might explain this variability. In a case-cohort study including the ...

journal_title：British journal of haematology

authors： Severinsen MT,Overvad K,Johnsen SP,Dethlefsen C,Madsen PH,Tjønneland A,Kristensen SR

更新日期：2010-04-01 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：:This study compared pulmonary function tests (PFTs) with cardiac, pancreatic and liver iron in 76 thalassemia major (TM) patients. Restrictive lung disease was observed in 16%, hyperinflation in 32% and abnormal diffusing capacity in 3%. While no patients met Global Initiative for Chronic Lung Disease criteria for air...

journal_title：British journal of haematology

authors： Sohn EY,Noetzli LJ,Gera A,Kato R,Coates TD,Harmatz P,Keens TG,Wood JC

更新日期：2011-10-01 00:00:00

abstract：:Differentiation therapy using retinoic acids (RAs) or 1alpha25-dihydroxyvitamin D3 (D3) is an attractive alternative to chemotherapy in acute myeloid leukaemia (AML) and myelodysplastic syndromes (MDS). However, with the exception of RA therapy for acute promyelocytic leukaemia (APL), RAs and D3 are not potent enough ...

journal_title：British journal of haematology

authors： Fenton SL,Drayson MT,Hewison M,Vickers E,Brown G,Bunce CM

更新日期：1999-05-01 00:00:00

abstract：:Lysosomal enzyme activities and isoenzyme profiles were measured in lymphoid and non-lymphoid leukaemic cells from childhood patients. High activities, especially of beta-hexosaminidase and alpha-mannosidase, were associated with leukaemic cells of myeloid or monocytic origin. Leukaemic cells from two children with ac...

journal_title：British journal of haematology

authors： Eden OB,Darbyshire P,Simpson RM,Besley GT,Moss S,Gentle T

更新日期：1985-01-01 00:00:00

abstract：:Flow cytometry was used to determine the percentage and number of circulating CD34+ cells in fetal blood from 100 pregnancies at 13-38 weeks gestation. When expressed as a percentage of the total number of lymphocytes, the proportion of CD34+ cells decreased exponentially from a mean of 11.1% (9.2 x 10(7)/l) at 13 wee...

journal_title：British journal of haematology

authors： Thilaganathan B,Nicolaides KH,Morgan G

更新日期：1994-07-01 00:00:00

abstract：:The expression of the VAV proto-oncogene in 57 patients with chronic myeloproliferative disease (CMD), B-cell acute lymphoblastic leukaemia (B-ALL) and B-cell non-Hodgkin Lymphoma (B-NHL), and 61 with B-cell chronic lymphocytic leukaemia (B-CLL) was analysed. VAV overexpression was observed in 19.5% of cases and 81% o...

journal_title：British journal of haematology

authors： Prieto-Sánchez RM,Hernández JA,García JL,Gutiérrez NC,San Miguel J,Bustelo XR,Hernández JM

更新日期：2006-06-01 00:00:00

abstract：:Hereditary hyperferritinaemia cataract syndrome is an autosomal dominant disorder caused by heterogeneous mutations of the iron regulatory element (IRE) in the ferritin l-chain mRNA. The mutations are rare and fast DNA scanning would facilitate diagnosis. The aim of the study was to compare the analytical performances...

journal_title：British journal of haematology

authors： Cremonesi L,Paroni R,Foglieni B,Galbiati S,Fermo I,Soriani N,Belloli S,Ruggeri G,Biasiotto G,Cazzola M,Ferrari F,Ferrari M,Arosio P

更新日期：2003-04-01 00:00:00

abstract：:A highly sensitive Y-microsatellite amplification system was developed for the detection of post bone marrow transplantation (BMT) chimaerism. The system is able to detect the DNA equivalent of a single male cell in a background of 10(5) female cells. For clinical applications it has the distinct advantage that the po...

journal_title：British journal of haematology

authors： Lo YM,Noakes L,Roux E,Jeannet M,Chapuis B,Fleming KA,Wainscoat JS

更新日期：1995-03-01 00:00:00

abstract：:Because the phagocytic function of non-stimulated human polymorphonuclear granulocytes (PMN) is impaired after incubation with either polynuclear Fe(III) or Fe(II), we decided to study lipid peroxidation of PMN and monocytes by these iron complexes. Lipid peroxidation was assessed by measuring thiobarbituric acid reac...

journal_title：British journal of haematology

authors： Hoepelman IM,Bezemer WA,van Doornmalen E,Verhoef J,Marx JJ

更新日期：1989-08-01 00:00:00

abstract：:Although individuals with sickle cell anaemia (SCA) have elevated baseline inflammation and endothelial activation, the acute phase response to maximal exercise has not been evaluated among children with SCA. We measured the acute phase response to maximal exercise testing for soluble vascular cell adhesion molecule (...

journal_title：British journal of haematology

authors： Liem RI,Onyejekwe K,Olszewski M,Nchekwube C,Zaldivar FP,Radom-Aizik S,Rodeghier MJ,Thompson AA

更新日期：2015-12-01 00:00:00