Erythrocyte adenosine deaminase: diagnostic value for Diamond-Blackfan anaemia.

abstract：:We report two patients with acute leukaemia who received emetogenic cytotoxic drugs and were on therapeutic doses of sodium valproate for epilepsy. Neither patient reported significant nausea nor vomited at any time during the chemotherapy, at times requiring no anti-emetic treatment whatsoever. We suggest that this a...

journal_title：British journal of haematology

authors： Aiken TC,Collin RC

更新日期：1995-04-01 00:00:00

abstract：:Kidney iron deposition measured by R2* (magnetic resonance imaging) MRI is posited to result from tubular reabsorption of filtered haemoglobin due to intravascular haemolysis. In chronically transfused sickle cell disease (SCD), R2* is elevated and positively correlated with lactate dehydrogenase (LDH). To account for...

journal_title：British journal of haematology

authors： Denton CC,Detterich JA,Coates TD,Wood JC

更新日期：2020-11-20 00:00:00

abstract：:The main objective of the present study was to determine the role of CD34+ cell subsets in the haemopoietic recovery of children undergoing peripheral blood stem cell transplantation. For this purpose, 38 leukaphereses from 33 children with malignancies mobilized with G-CSF were analysed. Using dual-colour flow cytome...

journal_title：British journal of haematology

authors： Gonzalez-Requejo A,Madero L,Díaz MA,Villa M,García-Escribano C,Balas A,Lillo R,García-Sanchez F,Benito A,Vicario JL

更新日期：1998-04-01 00:00:00

abstract：:Ernest Beutler was one of the preeminent haematologists of the last half of the 20th and the early 21st century. In a career that spanned six decades, his research interests included such diverse areas as red cell metabolism, blood preservation, glycolipid storage diseases, leukaemias and iron metabolism. Indeed, he w...

journal_title：British journal of haematology

authors： Beutler S,Beutler B

更新日期：2011-03-01 00:00:00

abstract：:Heterozygous factor XI (FXI) deficiency is sometimes associated with a significant bleeding tendency. Fresh frozen plasma of FXI concentrates are the mainstay of treatment in patients with a clear bleeding history, especially prior to surgery. However, these treatments are not completely free of risk. Furthermore, thr...

journal_title：British journal of haematology

authors： Castaman G,Ruggeri M,Rodeghiero F

更新日期：1996-07-01 00:00:00

abstract：:Plasma D-dimer was measured and compared with serum fibrinogen/fibrin degradation product levels (FDPs) in patients with disseminated intravascular coagulation (DIC) and other conditions associated with a hypercoagulable state. D-dimer (N less than 200 ng/ml) was elevated in all 43 patients with DIC, in 48 of 59 patie...

journal_title：British journal of haematology

authors： Wilde JT,Kitchen S,Kinsey S,Greaves M,Preston FE

更新日期：1989-01-01 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：:Haemophilia is an inherited disorder of clotting factor deficiencies resulting in musculoskeletal bleeding, including haemarthroses, leading to orthopaedic complications. The pathogenesis of haemophilic joint arthropathy continues to be explored and there is evidence to suggest that iron, cytokines, and neo angiogenes...

journal_title：British journal of haematology

authors： Acharya SS

更新日期：2012-01-01 00:00:00

abstract：:Inherited factor VII (FVII) deficiency is a rare autosomal recessive disorder associated with a bleeding tendency. We describe three patients with congenital FVII deficiency who have been treated with activated recombinant factor VII (rVIIa). Two patients had novel mutations and were treated prophylactically with 1.2 ...

journal_title：British journal of haematology

authors： Mathijssen NC,Masereeuw R,Verbeek K,Lavergne JM,Costa JM,van Heerde WL,Nováková IR

更新日期：2004-05-01 00:00:00

abstract：:The success of genetically engineered T cells that express chimeric antigen receptors (CARTs) has been a momentous step forward in harnessing the potent cancer fighting abilities of the immune system. The efficacy seen in relapsed/refractory (r/r) acute lymphoblastic leukaemia (ALL), not only by inducing remission, bu...

journal_title：British journal of haematology

authors： Orlowski RJ,Porter DL,Frey NV

更新日期：2017-04-01 00:00:00

abstract：:Recurrent large-scale somatic copy number alterations (SCNAs), and somatic point mutations can be analysed to stratify patients with chronic lymphocytic leukaemia (CLL) into distinct prognostic groups. To investigate the relationship between SCNAs and somatic mutations, we performed whole-exome sequencing and single n...

journal_title：British journal of haematology

authors： Zhou W,Goldin L,Wang M,McMaster ML,Jones K,Burdett L,Chanock SJ,Yeager M,Dean M,Caporaso NE

更新日期：2018-06-01 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：:We report a 22-year-old woman with acute, plasma refractory thrombotic thrombocytopenic purpura (TTP) in whom splenectomy led to consistent stabilization of platelet counts, but who showed complete inhibition of vonWillebrand factor-cleaving protease (VWF-cp) after 6 months of follow up. Persistent protease deficiency...

journal_title：British journal of haematology

authors： Langer F,Bergmann F,Budde U,Hegewisch-Becker S,Hossfeld DK

更新日期：2002-07-01 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：:Highly efficient systems remove toxic and pro-inflammatory haemoglobin (Hb) from the circulation and local sites of tissue damage. Macrophages are major Hb-clearing cells; CD163 was recently recognized as the specific haemoglobin-haptoglobin scavenger receptor (HSR). We show that dexamethasone strongly induced the spe...

journal_title：British journal of haematology

authors： Schaer DJ,Boretti FS,Schoedon G,Schaffner A

更新日期：2002-10-01 00:00:00

abstract：:During a survey of typical, dominant hereditary spherocytosis (HS) patients, we identified a frameshift mutation of the ankyrin gene in three unrelated probands. All three probands, who were from different ethnic backgrounds, suffered from severe HS requiring splenectomy. Analysis of both intragenic and flanking polym...

journal_title：British journal of haematology

authors： Gallagher PG,Ferreira JD,Costa FF,Saad ST,Forget BG

更新日期：2000-12-01 00:00:00

abstract：:The phenotypic expression and functional capacity of natural killer (NK) T-lymphocytes (E+, OKT3+) were analysed in a series of untreated patients with B-cell chronic lymphocytic leukaemia (B-CLL). The mean value of NK activity of B-CLL T-lymphocytes, tested against the K562 cell line, was significantly depressed (P l...

journal_title：British journal of haematology

authors： Foa R,Lauria F,Lusso P,Giubellino MC,Fierro MT,Ferrando ML,Raspadori D,Matera L

更新日期：1984-11-01 00:00:00

abstract：:Treatment with 75 microg/kg/d intravenous (i.v.) anti-D was compared with 50 microg/kg/d in a prospective randomized study of 27 RhD-positive, human immunodeficiency virus-negative, adult, acute, non-splenectomized patients with immune thrombocytopenic purpura (ITP) and platelet counts < or = 30 x 109/l. The higher do...

journal_title：British journal of haematology

authors： Newman GC,Novoa MV,Fodero EM,Lesser ML,Woloski BM,Bussel JB

更新日期：2001-03-01 00:00:00

abstract：:Genomic DNA from 106 cases of adult de novo acute myeloid leukaemia (AML) was screened by polymerase chain reaction (PCR) and gel electrophoresis for FLT3 internal tandem duplication (ITD) mutations within the juxtamembrane (JM) domain. FLT3 mutations were detected in 14 cases (13.2%) and occurred in FAB types M1 (4 o...

journal_title：British journal of haematology

authors： Abu-Duhier FM,Goodeve AC,Wilson GA,Gari MA,Peake IR,Rees DC,Vandenberghe EA,Winship PR,Reilly JT

更新日期：2000-10-01 00:00:00

abstract：:Pralatrexate inhibits folic acid metabolism, and preclinical studies have shown that it is cytotoxic to multiple myeloma cells. This phase 1 study investigated the safety and efficacy of pralatrexate in combination with bortezomib in adults with relapsed or refractory multiple myeloma. A standard 3 + 3 design was used...

journal_title：British journal of haematology

authors： Dunn TJ,Dinner S,Price E,Coutré SE,Gotlib J,Hao Y,Berube C,Medeiros BC,Liedtke M

更新日期：2016-04-01 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：: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：:Anti-cardiolipin antibody (aCL) measurement is only semi-reproducible, and current assays detect irrelevant as well as clinically significant antibodies. Factors found to influence results included the source of the enzyme-linked immunoabsorbent assay (ELISA) plate, and its pretreatment with solvents; the nature of th...

journal_title：British journal of haematology

authors： Kilpatrick DC

更新日期：1998-01-01 00:00:00

abstract：:Fanconi anaemia (FA) is an autosomal recessive disease strongly predisposing to bone marrow failure and acute myeloid leukaemia (AML). Four FA genes, corresponding to complementation groups A, C, F and G, have been cloned, but the molecular functions of the corresponding proteins are unknown. The high risk of AML in F...

journal_title：British journal of haematology

authors： Xie Y,de Winter JP,Waisfisz Q,Nieuwint AW,Scheper RJ,Arwert F,Hoatlin ME,Ossenkoppele GJ,Schuurhuis GJ,Joenje H

更新日期：2000-12-01 00:00:00

abstract：:Association between HLA haplotypes and long survival was investigated in 116 children with acute lymphoblastic leukaemia. It was found that patients with A2 B12 and/or A2 B40 haplotypes survived longer than patients wtihout these two haplotypes. Since all children were treated with transfer factor obtained from their ...

journal_title：British journal of haematology

authors： De Bruyère M,Cornu G,Heremans-Bracke T,Malchaire J,Sokal G

更新日期：1980-02-01 00:00:00

abstract：:Iron uptake and storage in peripheral blood leucocytes has been studies to test the hypothesis that the basic lesion in idiopathic haemochromatosis resides in a failure of mononuclear phagocytic cells to store iron. In seven fully treated cases of this disease there was no evidence of any abnormality in iron uptake, s...

journal_title：British journal of haematology

authors： Jacobs A,Summers MR

更新日期：1981-12-01 00:00:00

abstract：:The determinants of steady state haemoglobin levels in sickle cell-haemoglobin C (SC) disease were investigated by measuring routine haematological and biochemical indices, red cell survival, oxygen affinity, pitted erythrocytes, and red cell and plasma volumes in 31 adult patients (15 male; 16 female). Red cell survi...

journal_title：British journal of haematology

authors： Bannerman RM,Serjeant B,Seakins M,England JM,Serjeant GR

更新日期：1979-09-01 00:00:00

abstract：:The polymerase chain reaction (PCR) assay was used to detect human parvovirus B19 DNA in 38 blood products and start plasma pools from five different manufacturers. The products examined were albumin, factor VIII, intravenous (i.v.) and intramuscular (i.m) immunoglobulin batches. The majority of pools from all the man...

journal_title：British journal of haematology

authors： Saldanha J,Minor P

更新日期：1996-06-01 00:00:00

abstract：:In a phase II study, 12 patients with a myelodysplastic syndrome (MDS) and anaemia (nine transfusion-dependent) were treated with recombinant human erythropoietin (rHuEpo) to assess the therapeutic effect on erythropoiesis and on transfusion requirement. Patients with a low risk of developing acute leukaemia were incl...

journal_title：British journal of haematology

authors： van Kamp H,Prinsze-Postema TC,Kluin PM,den Ottolander GJ,Beverstock GC,Willemze R,Fibbe WE

更新日期：1991-08-01 00:00:00

abstract：:Physiological parameters such as pH and oxygen tension probably play significant roles in the regulation of haemopoiesis in the bone marrow microenvironment, but these roles have yet to be characterized in detail. We have found that changes in culture pH (0.2 units) can cause significant changes in the culture composi...

journal_title：British journal of haematology

authors： McAdams TA,Miller WM,Papoutsakis ET

更新日期：1998-11-01 00:00:00