Evidence for a continuous decline in haemopoietic cell function from birth: application to evaluating bone marrow failure in children.

abstract：:Sixty patients with von Willebrand's disease belonging to 34 unrelated families were classified into the various types of the disease by analysis of the multimer patterns of von Willebrand factor. Type I disease was observed in 62% of the families, a finding similar to the recently published British and Swedish series...

journal_title：British journal of haematology

authors： Berliner SA,Seligsohn U,Zivelin A,Zwang E,Sofferman G

更新日期：1986-03-01 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：: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：: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 use of peripheral blood stem cells instead of bone marrow as the source of haemopoietic cells for allogeneic transplantation is being increasingly explored. We have analysed data from 17 normal donors who underwent stem cell mobilization for allogeneic transplantation with an identical protocol using G-CSF at a do...

journal_title：British journal of haematology

authors： Miflin G,Charley C,Stainer C,Anderson S,Hunter A,Russell N

更新日期：1996-11-01 00:00:00

abstract：:A 33-year-old man presented with hepatosplenomegaly and lymphadenopathy. Bone marrow findings were consistent with Philadelphia-positive accelerated-phase chronic granulocytic leukaemia (CGL). Lymph node histology and immunhistochemistry were diagnostic of T lymphoblastic lymphoma. Molecular studies on lymph node DNA ...

journal_title：British journal of haematology

authors： Jacob A,Rowlands DC,Patton N,Holmes JA

更新日期：1994-10-01 00:00:00

abstract：:Interleukin-10 (IL-10) is one of the cytokines implicated in the pathogenesis of diffuse large B-cell lymphoma (DLBCL) in which it acts as auto/paracrine growth factor for lymphoma growth. T-cell non-Hodgkin lymphoma (NHL) is a heterogeneous disease, the biological basis of which is not fully understood. Some evidence...

journal_title：British journal of haematology

authors： Lee JJ,Kim DH,Lee NY,Sohn SK,Kim JG,Kim HJ,Do YR,Park YH

更新日期：2007-05-01 00:00:00

abstract：:Many immune dysfunctions participate in immune thrombocytopenia (ITP) pathogenesis, including numeric and functional defects in suppressor T (Ts) cells and immune-regulation abnormalities in mesenchymal stem cells (MSCs). Recent studies showed that MSCs can promote Ts cell differentiation. Thus, we compared the Ts cel...

journal_title：British journal of haematology

authors： Li H,Guan Y,Sun B,Dou X,Liu X,Xue F,Fu R,Zhang L,Yang R

更新日期：2020-07-16 00:00:00

abstract：:We report a patient with poor-prognosis myelodysplastic syndrome (MDS) after successful treatment of lymphoma, who was given an allogeneic BMT, engrafted and achieved complete remission, but later had a relapse of his MDS with complete disappearance of donor haemopoiesis. After two episodes of CMV pneumonia and contin...

journal_title：British journal of haematology

authors： Beguin Y,Collignon J,Laurent C,Fillet G

更新日期：1996-09-01 00:00:00

abstract：:The pattern of distribution of von Willebrand factor (VWF) in relatively large sheets of rat aortic endothelial cells (EC) obtained by the Häutchen technique were analysed by immunocytochemistry and light microscopy. EC were examined pre and post administration of a procoagulant mixture of factor Xa (F.Xa) and phospho...

journal_title：British journal of haematology

authors： Senis YA,Richardson M,Tinlin S,Maurice DH,Giles AR

更新日期：1996-04-01 00:00:00

abstract：:The records of 121 children presenting with acute lymphoblastic leukaemia between 1969 and 1982 were reviewed. The overall relapse free survival rate was 50%. However, girls presenting with a total white blood cell count of 20 X 10(9)/l or less (35% of all patients) had a particularly favourable prognosis with an 80% ...

journal_title：British journal of haematology

authors： Stockley RJ,Ahlquist PY,Mott MG

更新日期：1983-10-01 00:00:00

abstract：:Juvenile haemochromatosis or haemochromatosis type 2 is a rare autosomal recessive disorder which causes iron overload at a young age, affects both sexes equally and is characterized by a prevalence of hypogonadism and cardiopathy. Patients with haemochromatosis type 2 have been reported in different ethnic groups. Li...

journal_title：British journal of haematology

authors： De Gobbi M,Roetto A,Piperno A,Mariani R,Alberti F,Papanikolaou G,Politou M,Lockitch G,Girelli D,Fargion S,Cox TM,Gasparini P,Cazzola M,Camaschella C

更新日期：2002-06-01 00:00:00

abstract：:The expression of Fc(IgG) and C3b membrane receptors by granulocytes and their precursors was examined in 23 cases of megaloblastic anaemia which were graded I-III according to morphological severity. Fractionated bone marrow and peripheral blood granulocyte receptors were assessed by rosette formation with optimally ...

journal_title：British journal of haematology

authors： Scott CS,Bynoe AG,Roberts BE,Hough D

更新日期：1982-11-01 00:00:00

abstract：:The prognosis of acute lymphoblastic leukaemia (ALL) in adults is poor compared with children in terms of complete remission (CR) and leukaemia-free survival. In children in vitro resistance of leukaemic cells to various cytotoxic agents is an independent poor prognostic marker, but the relevance of in vitro drug resi...

journal_title：British journal of haematology

authors： Maung ZT,Reid MM,Matheson E,Taylor PR,Proctor SJ,Hall AG

更新日期：1995-09-01 00:00:00

abstract：:Although 5-fluorouracil (FUra) has been the drug of choice for the treatment of patients with advanced colorectal carcinoma, the response rates are in the range of 15% and the median survival times do not exceed 9 months. Interferon alpha (IFN alpha) has limited antitumour activity in this disease. Recent clinical tri...

journal_title：British journal of haematology

authors： Rustum YM

更新日期：1991-10-01 00:00:00

abstract：:Down syndrome (DS) children are at an increased risk of developing myelodysplasia and acute myeloid leukaemia (AML). We retrospectively analysed the population-based data on 81 children with myeloid leukaemia of Down syndrome (ML-DS) from the UK National Registry of Childhood Tumours and experience in the Medical Rese...

journal_title：British journal of haematology

authors： Rao A,Hills RK,Stiller C,Gibson BE,de Graaf SS,Hann IM,O'Marcaigh A,Wheatley K,Webb DK

更新日期：2006-03-01 00:00:00

abstract：:Relapse of childhood acute lymphoblastic leukaemia (ALL) involving the eye is a rare but challenging problem. Twenty cases occurred in patients treated on the Medical Research Council United Kingdom Acute Lymphoblastic Leukaemia XI and ALL97 trials between 1991 and 2001, representing 2.2% of ALL relapses. Seventeen oc...

journal_title：British journal of haematology

authors： Somervaille TC,Hann IM,Harrison G,Eden TO,Gibson BE,Hill FG,Mitchell C,Kinsey SE,Vora AJ,Lilleyman JS,MRC Childhood Leukaemia Working Party.

更新日期：2003-04-01 00:00:00

abstract：:The prognostic impact of bone marrow biopsy (BMB) histology and CD34 immunoreactivity was compared with that of the more conventional parameters (the FAB diagnosis, peripheral blood values, percentage of BM blasts and some common prognostic scores) in 100 MDS patients. Statistical correlations among the cytological, h...

journal_title：British journal of haematology

authors： Oriani A,Annaloro C,Soligo D,Pozzoli E,Cortelezzi A,Lambertenghi Deliliers G

更新日期：1996-02-01 00:00:00

abstract：:MPLW515L/K and JAK2V617F can co-exist in myelofibrosis with myeloid metaplasia (MMM). The chronology of clonal emergence was studied in three such cases using serially stored bone marrow. At diagnosis, a major MPL515 mutant clone was accompanied by a minor JAK2V617F clone in all three instances. At 25 time points over...

journal_title：British journal of haematology

authors： Lasho TL,Pardanani A,McClure RF,Mesa RA,Levine RL,Gilliland DG,Tefferi A

更新日期：2006-12-01 00:00:00

abstract：:Second primary malignancies have long been associated with chronic lymphocytic leukaemia (CLL). We assessed secondary tumour samples from CLL and control patients for the presence of human papilloma virus (HPV). 132 CLL patients with 44 second malignancies were compared to a matched randomly-identified control populat...

journal_title：British journal of haematology

authors： Flynn JM,Andritsos L,Lucas D,Byrd JC

更新日期：2010-05-01 00:00:00

abstract：:Minimal residual disease (MRD) assessment is a powerful prognostic factor for determining the risk of relapse in childhood acute lymphoblastic leukaemia (ALL). In this Swedish multi-centre study of childhood ALL diagnosed between 2002 and 2006, the MRD levels were analysed in 726 follow-up samples in 228 children usin...

journal_title：British journal of haematology

authors： Thörn I,Forestier E,Botling J,Thuresson B,Wasslavik C,Björklund E,Li A,Lindström-Eriksson E,Malec M,Grönlund E,Torikka K,Heldrup J,Abrahamsson J,Behrendtz M,Söderhäll S,Jacobsson S,Olofsson T,Porwit A,Lönnerholm G,R

更新日期：2011-03-01 00:00:00

abstract：:The incidence, characteristics and risk factors for the development of osteonecrosis of the jaw (ONJ) were evaluated among 303 myeloma patients. Only patients who received bisphosphonates developed ONJ (28/254; 11%). Zoledronic acid produced 9.5-fold greater risk for developing ONJ than pamidronate alone (P = 0.042) a...

journal_title：British journal of haematology

authors： Zervas K,Verrou E,Teleioudis Z,Vahtsevanos K,Banti A,Mihou D,Krikelis D,Terpos E

更新日期：2006-09-01 00:00:00

abstract：:In previous trials, the orally active iron chelator deferiprone (L1) has been associated with sporadic agranulocytosis, milder forms of neutropenia and other side-effects. To determine the incidence of these events, we performed a multicentre prospective study of the chelator. Blood counts were performed weekly, and c...

journal_title：British journal of haematology

authors： Cohen AR,Galanello R,Piga A,Dipalma A,Vullo C,Tricta F

更新日期：2000-02-01 00:00:00

abstract：:The activities of glucose-6-phosphate dehydrogenase (D-glucose-6-phosphate: NADP oxidoreductase, G6PD), 6 phosphate glucono dehydrogenase (6 phospho-D-gluconate: NADP oxidoreductase, 6PGD) lactate dehydrogenase (D-lactate: NAD oxidoreductase, LDH), glutamate oxaloacetate transaminase (L-aspartate: 2-oxo-glutarate amin...

journal_title：British journal of haematology

authors： Ramot B,Brok-Simoni F,Chweidan E,Ashkenazi YE

更新日期：1976-09-01 00:00:00

abstract：:Alpha haemoglobin stabilising protein (AHSP) binds free alpha-globin chains and plays an important role in the protection of red cells, particularly during beta-thalassaemia. Murine ASHP was discovered as a GATA-1 target gene and human AHSP is directly regulated by GATA-1. More recently, AHSP was rediscovered as a hig...

journal_title：British journal of haematology

authors： Keys JR,Tallack MR,Hodge DJ,Cridland SO,David R,Perkins AC

更新日期：2007-01-01 00:00:00

abstract：:The combination of tumour necrosis factor alpha (TNF alpha) and gamma-interferon induced transcription of class I HLA genes in chronic myelogenous leukaemia (CML) cell lines through the formation of a complex between nuclear proteins and the transcriptional enhancers associated with these genes. Although gamma-interfe...

journal_title：British journal of haematology

authors： Seong D,Sims S,Johnson E,Lyding J,Lopez A,Garovoy M,Talpaz M,Kantarjian H,Lopez-Berestein G,Reading C

更新日期：1991-07-01 00:00:00

abstract：:The in vitro effects of a range of concentrations of cyclophosphamide, doxorubicin, actinomycin D, cytarabine and methotrexate on neutrophil phagocytosis of C. albicans was studied. The reduction in phagocytic index (PI) was inversely proportional to the dilution of the drug and there was some inhibition of phagocytos...

journal_title：British journal of haematology

authors： Davies JE,Whittaker JA,Khurshid M

更新日期：1976-01-01 00:00:00

abstract：:Thrombocytopenia absent radii (TAR) syndrome is clearly defined by the combination of radial aplasia and reduced platelet counts. The genetics of TAR syndrome has recently been resolved and comprises a microdeletion on Chromosome 1 including the RBM8A gene and a single nucleotide polymorphism (SNP) either at the 5' un...

journal_title：British journal of haematology

authors： Manukjan G,Bösing H,Schmugge M,Strauß G,Schulze H

更新日期：2017-11-01 00:00:00

abstract：:We previously demonstrated that multitransfused patients with severe aplastic anaemia (SAA) exhibit high numbers of alloreactive cytotoxic T lymphocyte precursors directed against their HLA identical siblings. In this study a group of patients who had received multiple blood transfusions for SAA, other haematological ...

journal_title：British journal of haematology

authors： Kaminski ER,Hows JM,Goldman JM,Batchelor JR

更新日期：1992-05-01 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