Patients with chronic lymphocytic leukaemia and clonal deletion of both 17p13.1 and 11q22.3 have a very poor prognosis.

abstract：:In this study we show that in vitro cultured human polyclonal NK cell lines and clones express the Ki-1/CD30 Hodgkin-associated antigen, identified by the BER-H2 monoclonal antibody. The percentage of BER-H2+ cells ranged from 19% to 67% in five polyclonal NK cell lines and was 31% and 20% in two NK clones. The intens...

journal_title：British journal of haematology

authors： Cambiaggi A,Cantoni C,Marciano S,De Totero D,Pileri S,Tazzari PL,Stein H,Ferrini S

更新日期：1993-10-01 00:00:00

abstract：:We have characterized the molecular defect in two families with severe factor VII (FVII) deficiency. In family I, the proband was found to be homozygous for a novel 18 bp deletion in exon 8 (g.10896-10913del) resulting in the in-frame deletion of six amino acids in the serine protease domain. Molecular modelling sugge...

journal_title：British journal of haematology

authors： Gomez K,Laffan MA,Kemball-Cook G,Pasi J,Layton M,Singer JD,Tuddenham EG,McVey JH

更新日期：2004-07-01 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：:Risk factors for acute graft-versus-host disease (GvHD) remain controversial. We performed uni- and multivariate statistical analyses on a series of 37 patients receiving a non-depleted allogeneic bone marrow transplant from an HLA-identical sibling donor for a haematological malignancy, in order to identify risk fact...

journal_title：British journal of haematology

authors： Bagot M,Mary JY,Heslan M,Kuentz M,Cordonnier C,Vernant JP,Dubertret L,Levy JP

更新日期：1988-12-01 00:00:00

abstract：:Allogeneic stem cell transplantation (alloSCT) is a curative procedure for myelofibrosis. Elderly people are mainly affected, limiting the feasibility of myeloablative regimens. The introduction of reduced-intensity conditioning (RIC) made alloSCT feasible for older patients. Nevertheless, the incidence of myelofibros...

journal_title：British journal of haematology

authors： Mannina D,Zabelina T,Wolschke C,Heinzelmann M,Triviai I,Christopeit M,Badbaran A,Bonmann S,von Pein UM,Janson D,Ayuk F,Kröger N

更新日期：2019-08-01 00:00:00

abstract：:Mature (peripheral) T-cell lymphoma (PTCL) other than anaplastic large cell lymphoma is a heterogeneous group of diseases and exceedingly rare in children and adolescents. Survival rates range between 46% and 85%. This study reports the disease characteristics, treatment and outcome of all patients with the diagnosis ...

journal_title：British journal of haematology

authors： Kontny U,Oschlies I,Woessmann W,Burkhardt B,Lisfeld J,Salzburg J,Janda A,Attarbaschi A,Niggli F,Zimmermann M,Reiter A,Klapper W

更新日期：2015-03-01 00:00:00

abstract：:Cardiopulmonary bypass (CPB) induces a bleeding defect which leads to enhanced blood loss. A double-blind study was carried out comparing aprotinin with placebo in patients undergoing re-operation for heart valve replacement. The results confirm that aprotinin is effective at reducing such loss. In the placebo treated...

journal_title：British journal of haematology

authors： Orchard MA,Goodchild CS,Prentice CR,Davies JA,Benoit SE,Creighton-Kemsford LJ,Gaffney PJ,Michelson AD

更新日期：1993-11-01 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：:The application of ultraviolet B (UVB) radiation has been proposed as a new technology to decrease immunogenicity of leucocytes in platelet transfusions. UV radiation also induces platelet aggregation, which occurs most effectively at wavelengths between 240 and 280 nm and falls off sharply above 300 nm. In order to m...

journal_title：British journal of haematology

authors： van Prooijen HC,van Marwijk Kooy M,van Weelden H,Aarts-Riemens MI,Borghuis L,Akkerman JW

更新日期：1990-08-01 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：:Antithrombin III (AT III) activity has been measured in 9669 healthy blood donors (5525 male and 4144 female). The distribution of AT III is approximately 'normal' with mean 105.6 IU/dl and standard deviation 11.2; however, definite age and sex related variations are evident. Pre-menopausal females have lower mean AT ...

journal_title：British journal of haematology

authors： Tait RC,Walker ID,Islam SI,McCall F,Conkie JA,Mitchell R,Davidson JF

更新日期：1993-07-01 00:00:00

abstract：:We describe five adult patients with sickle cell anaemia (SS) who developed clinical, radiological and histological evidence of splenic regrowth while receiving regular blood transfusions. Five patients, all homozygous SS, range 23-34 years, were commenced on hypertransfusion therapy. Three patients were transfused be...

journal_title：British journal of haematology

authors： Campbell PJ,Olatunji PO,Ryan KE,Davies SC

更新日期：1997-01-01 00:00:00

abstract：:Between 1984 and 1992, 21 patients with chronic myeloid leukaemia (CML) in chronic phase (CP) were treated with high-dose chemotherapy (or chemoradiotherapy) followed by autografting with unmanipulated peripheral blood stem cells (PBSC). 12 of these patients survive at a median of 82 months from the time of autografti...

journal_title：British journal of haematology

authors： Hoyle C,Gray R,Goldman J

更新日期：1994-01-01 00:00:00

abstract：:Haem biosynthesis is the most important destination for absorbed iron, hence it can be hypothesized that iron absorption regulation should be integrated with haem metabolism. As an initial step to test this hypothesis, the effect on iron absorption of Tin-mesoporphyrin (SnMP), inhibitor of haem oxygenase, altering hae...

journal_title：British journal of haematology

authors： Laftah AH,Raja K,Simpson RJ,Peters TJ

更新日期：2003-07-01 00:00:00

abstract：:Mantle cell lymphoma (MCL) is a heterogenic non-Hodgkin lymphoma entity, with a median survival of about 5 years. In 2008 we reported the early - based on the median observation time of 4 years - results of the Nordic Lymphoma Group MCL2 study of frontline intensive induction immunochemotherapy and autologous stem cel...

journal_title：British journal of haematology

authors： Geisler CH,Kolstad A,Laurell A,Jerkeman M,Räty R,Andersen NS,Pedersen LB,Eriksson M,Nordström M,Kimby E,Bentzen H,Kuittinen O,Lauritzsen GF,Nilsson-Ehle H,Ralfkiaer E,Ehinger M,Sundström C,Delabie J,Karjalainen-Lindsb

更新日期：2012-08-01 00:00:00

abstract：:Flow cytometry represents an alternative method to agglutination assays for the accurate quantification of mixed field populations of erythrocytes observed after bone marrow transplantation. Murine monoclonal antibodies directed against the blood group ABH antigens were selected and processed in order to prepare ready...

journal_title：British journal of haematology

authors： Blanchard D,Bruneau V,Bernard D,Germond-Arnoult F,Gourbil A,David B,Muller JY

更新日期：1995-04-01 00:00:00

abstract：:Mastocytosis is an uncommon disease characterized by a proliferation of tissue mast cells involving various organs, particularly the bone marrow. Though rare, the association of mastocytosis and haematological malignancies is well established. However, the frequency of mastocytosis reported in patients with essential ...

journal_title：British journal of haematology

authors： Le Tourneau A,Gaulard P,D'Agay MF,Vainchencker W,Cadiou M,Devidas A,Haioun C,Clauvel JP,Audouin J,Diebold J

更新日期：1991-09-01 00:00:00

abstract：:Inhibition of apoptosis (genetically programmed active cell death) by p210 BCR-ABL expression is a mechanism that might contribute to clonal expansion in chronic myeloid leukaemia (CML). Since cell death following exposure to ionizing radiation and many chemotherapeutic agents can occur by the apoptotic pathway, inhib...

journal_title：British journal of haematology

authors： Amos TA,Lewis JL,Grand FH,Gooding RP,Goldman JM,Gordon MY

更新日期：1995-10-01 00:00:00

abstract：:S-Methylation by thiopurine methyltransferase (TPMT) is an important route of metabolism for the thiopurine drugs. About one in 300 individuals are homozygous for a TPMT mutation associated with very low enzyme activity and severe myelosuppression if treated with standard doses of drug. To validate the use of molecula...

journal_title：British journal of haematology

authors： Coulthard SA,Rabello C,Robson J,Howell C,Minto L,Middleton PG,Gandhi MK,Jackson G,McLelland J,O'Brien H,Smith S,Reid MM,Pearson AD,Hall AG

更新日期：2000-09-01 00:00:00

abstract：:Bone involvement is the commonest clinical manifestation of sickle cell disease both in the acute setting such as painful vaso-occlusive crises, and as a source of chronic, progressive disability such as avascular necrosis. Management of these problems is often difficult because of the diagnostic imprecision of most l...

journal_title：British journal of haematology

authors： Almeida A,Roberts I

更新日期：2005-05-01 00:00:00

abstract：:The process of tumorigenesis is frequently associated with resistance to growth inhibition by physiological regulators of normal cells. Murine macrophage-like cell lines BAC1.2F5, RAW264, J774.1A and PU5/1.8 were resistant to growth inhibition by bacterial lipopolysaccharide (LPS) and pertussis toxin, agents that bloc...

journal_title：British journal of haematology

authors： Xie Y,von Gavel S,Cassady AI,Stacey KJ,Dunn TL,Hume DA

更新日期：1993-07-01 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：:To determine the effects of L-arginine (L-Arg) supplementation on nitric oxide metabolite (NOx) production, oral L-Arg was given to normal controls, sickle cell disease (SCD) patients at steady state and SCD patients hospitalized with a vaso-occlusive crisis (VOC). L-Arg (0.1 g/kg) increased NOx formation by 18.8 +/- ...

journal_title：British journal of haematology

authors： Morris CR,Kuypers FA,Larkin S,Sweeters N,Simon J,Vichinsky EP,Styles LA

更新日期：2000-11-01 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：:Karyotypic analysis was performed in a total of 69 patients with well-characterized idiopathic myelofibrosis. Karyotypic abnormalities were detected in 46% of cases examined during the chronic phase (29/63); with three abnormalities, del(13q), del(20q) and partial trisomy 1q, accounting for 75% of all abnormalities at...

journal_title：British journal of haematology

authors： Reilly JT,Wilson G,Barnett D,Watmore A,Potter A

更新日期：1994-11-01 00:00:00

abstract：:Terminal B-cell differentiation is a multi-step process, from short-lived plasmablasts to mature long-lived plasma cells (PC). The anti-apoptotic Bcl-2 family member Bfl-1/A1 plays a critical role in the survival of mature B cells. However, its potential involvement at the later stages of B-cell development remains hi...

journal_title：British journal of haematology

authors： Tarte K,Jourdan M,Veyrune JL,Berberich I,Fiol G,Redal N,Shaughnessy J Jr,Klein B

更新日期：2004-05-01 00:00:00

abstract：:Gaucher disease (GD) is an autosomal recessive inherited defect of the lysosomal enzyme glucocerebrosidase (GluCerase) that leads to glucosylceramide (GluCer) accumulation. We previously demonstrated the existence of imbalances in certain lymphocyte populations in GD patients. We now show that GluCerase-deficient mono...

journal_title：British journal of haematology

authors： Balreira A,Lacerda L,Miranda CS,Arosa FA

更新日期：2005-06-01 00:00:00

abstract：:Patients with solid tumours undergoing high-dose chemotherapy with autologous bone marrow transplantation use an average of 10 units of packed red blood cells (PRBC) while awaiting haemopoietic reconstitution. They are also known to have inappropriately low endogenous erythropoietin levels for their degree of anaemia....

journal_title：British journal of haematology

authors： Ayash LJ,Elias A,Hunt M,Demetri G,Wheeler C,Tepler I,Schwartz G,Mazanet R,Reich E,McCauley M

更新日期：1994-05-01 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：:Aggregation, exposure of procoagulant phospholipids and shedding of microparticles are platelet responses that depend on activating conditions. To determine how these different responses are interconnected, we simultaneously measured fibrinogen (Fg) binding and aminophospholipid exposure on activated platelets by mean...

journal_title：British journal of haematology

authors： Bachelot-Loza C,Badol P,Brohard-Bohn B,Fraiz N,Cano E,Rendu F

更新日期：2006-05-01 00:00:00