Investigation of a large kindred with type IIB von Willebrand's disease, dominant inheritance and age-dependent thrombocytopenia.

abstract：:Tumour lysis syndrome (TLS) in plasma cell dyscrasias is extremely rare. TLS has been described in eight cases of multiple myeloma undergoing high-dose therapy with autologous stem cell transplant (ASCT). Recently, clinical trials of intensive chemotherapy followed by autologous or allogeneic stem cell support has bee...

journal_title：British journal of haematology

authors： Akasheh MS,Chang CP,Vesole DH

更新日期：1999-11-01 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：:A new glucose-6-phosphate dehydrogenase variant detected in an Italian man from the Po delta is described and designated as G6PD Modena. Biochemical characterization of the variant enzyme revealed an activity 21% of normal, a slow electrophoretic mobility, increased Km value for NADP, decreased Km value for G6P and a ...

journal_title：British journal of haematology

authors： Cappellini MD,Sampietro M,Toniolo D,Carandina G,Pittalis S,Martinez di Montemuros F,Tavazzi D,Fiorelli G

更新日期：1994-05-01 00:00:00

abstract：:Sickle cell disease (SCD) affects approximately 100,000 people in the US, 12,500 in the UK, and millions worldwide. SCD is typified by painful vaso-occlusive episodes, haemolytic anaemia and organ damage. A secondary complication is infection, which can be bacterial, fungal or viral. Universal newborn screening, routi...

journal_title：British journal of haematology

authors： Sobota A,Sabharwal V,Fonebi G,Steinberg M

更新日期：2015-09-01 00:00:00

abstract：:Molecular genetic studies of lymphoma have led to refinements in disease classification in the most recent World Health Organization update. Nevertheless, a 'one-size-fits-most' treatment strategy based on morphology remains widely used for lymphoma despite significant molecular heterogeneity within histopathologicall...

journal_title：British journal of haematology

authors： El-Galaly TC,Cheah CY,Villa D

更新日期：2019-08-01 00:00:00

abstract：:Among 80 hereditary spherocytosis (HS) kindreds studied using denaturing electrophoretic separation of solubilized eythrocyte membrane proteins, we recognized three prominent subsets: HS with isolated spectrin deficiency, HS with combined spectrin and ankyrin deficiency, and HS with band 3 deficiency These three subse...

journal_title：British journal of haematology

authors： Dhermy D,Galand C,Bournier O,Boulanger L,Cynober T,Schismanoff PO,Bursaux E,Tchernia G,Boivin P,Garbarz M

更新日期：1997-07-01 00:00:00

abstract：:Any influence of G-CSF on eosinophils is mostly negative, although reports which have studied this relationship are few with varied results. The aim of this study was to investigate the influence of G-CSF administration to healthy subjects on eosinophils in peripheral blood. Blood eosinophil counts, serum levels of eo...

journal_title：British journal of haematology

authors： Karawajczyk M,Höglund M,Ericsson J,Venge P

更新日期：1997-02-01 00:00:00

abstract：:The issue of whether, in patients affected by myelodysplastic syndromes (MDS), haematological response to cytokines, particularly to recombinant human erythropoietin (rHuEpo), is a phenomenon related to the stimulation of normal haemopoietic cells or to the differentiation of cells belonging to the abnormal clone rema...

journal_title：British journal of haematology

authors： Rigolin GM,Porta MD,Bigoni R,Cavazzini F,Ciccone M,Bardi A,Cuneo A,Castoldi G

更新日期：2002-12-01 00:00:00

abstract：:Very Late Antigen-4 (VLA-4, α4β1-integrin, ITGA4) orchestrates cell-cell and cell-endothelium adhesion. Given the proposed role of VLA-4 in sickle cell disease (SCD) pathophysiology, we evaluated the ability of the VLA-4 blocking antibody natalizumab to inhibit SCD blood cell adhesion. Natalizumab recognized surface V...

journal_title：British journal of haematology

authors： White J,Krishnamoorthy S,Gupta D,Lancelot M,Moore N,Sarnaik S,Hobbs WE 2nd,Light DR,Hines P

更新日期：2016-09-01 00:00:00

abstract：:This study reports the characterization of a spontaneous lymphoblastoid cell line (LCL) raised from the peripheral blood of a patient with Kostmann's congenital neutropenia. The LCL was composed of EBV-infected polyclonal B cells and displayed surface markers and pattern of growth in vitro typical of normal LCLs. The ...

journal_title：British journal of haematology

authors： Corcione A,Roncella S,Cutrona G,Mori PG,Ferrarini M,Pistoia V

更新日期：1993-12-01 00:00:00

abstract：:Intrinsic factor (IF) and cobalamin-R-binding protein (R-binder) linked to polyacrylamide beads were used to absorb cobalamins from solutions and serum extracts. Both binding agents were equally effective in removing [57Co]B12 from aqueous solution. IF was more effective than R-binder in removing [57Co]B12 added to a ...

journal_title：British journal of haematology

authors： Muir M,Chanarin I

更新日期：1983-08-01 00:00:00

abstract：:Translocations involving the MLL gene on the chromosome 11 (11q23) are frequently observed in acute leukaemia. The detection of this genetic change has a unique significance as a result of its implication of poor prognosis. To reveal the utility of fluorescence in situ hybridization (FISH) in detecting the MLL translo...

journal_title：British journal of haematology

authors： Kim HJ,Cho HI,Kim EC,Ko EK,See CJ,Park SY,Lee DS

更新日期：2002-12-01 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 aim of the present work was to investigate the effect of selective beta-I-blockade on the exchangeable splenic platelet pool (ESPP). Therefore, 50 mg of metoprolol (a selective beta-I-receptor blocking agent) was given by mouth to three groups of subjects: (1) 15 healthy volunteers, (2) five asplenic subjects, and...

journal_title：British journal of haematology

authors： Kutti J,Fredén K,Melberg PE,Lundborg P

更新日期：1977-10-01 00:00:00

abstract：:Diamond-Blackfan anaemia (DBA) is a constitutional pure red cell aplasia presenting in early childhood. In some patients, neutropenia and/or thrombocytopenia have also been observed during the course of the disease. We have followed 28 patients with steroid-refractory DBA for up to 13 years with serial peripheral bloo...

journal_title：British journal of haematology

authors： Giri N,Kang E,Tisdale JF,Follman D,Rivera M,Schwartz GN,Kim S,Young NS,Rick ME,Dunbar CE

更新日期：2000-01-01 00:00:00

abstract：:Demand for anticoagulation management is increasing due to an expansion of clinical indications for therapy. One possible model of care to meet demand is patient self-management (PSM), beneficial to patients who need control over their condition. This study aimed to determine the cost and cost-effectiveness of PSM of ...

journal_title：British journal of haematology

authors： Jowett S,Bryan S,Murray E,McCahon D,Raftery J,Hobbs FD,Fitzmaurice D

更新日期：2006-09-01 00:00:00

abstract：:Patients with sickle cell disease (SCD) with high fetal haemoglobin (HbF) tend to have a lower incidence of complications and longer survival due to inhibition of deoxyhaemoglobin S (HbS) polymerisation by HbF. HbF-containing cells, namely F cells, are strongly influenced by genetic factors. We measured the percentage...

journal_title：British journal of haematology

authors： Urio F,Nkya S,Rooks H,Mgaya JA,Masamu U,Zozimus Sangeda R,Mmbando BP,Brumat M,Mselle T,Menzel S,Luzzatto L,Makani J

更新日期：2020-10-19 00:00:00

abstract：:We studied the lysis in vitro of group A red cells by IgG anti-A. IgG anti-A, which strongly lysed A red cells from adults, did not lyse A red cells from cord blood, if fresh cord serum from a child with blood group AB was used as a source of complement. In cases of haemolytic disease of the newborn due to A-O or B-O ...

journal_title：British journal of haematology

authors： Brouwers HA,Overbeeke MA,Huiskes E,Bos MJ,Ouwehand WH,Engelfriet CP

更新日期：1988-03-01 00:00:00

abstract：:Recent studies concerning the numbers of circulating clonal B cells in patients with multiple myeloma (MM) have reported conflicting data regarding the exact levels of clonal B cells and the existence of clonal cells in the CD34 compartment. In this report we show that high numbers of clonal cells with a phenotype of ...

journal_title：British journal of haematology

authors： Rasmussen T,Kastrup J,Knudsen LM,Johnsen HE

更新日期：1999-04-01 00:00:00

abstract：:A primary analysis of the ASPIRE study found that the addition of carfilzomib to lenalidomide and dexamethasone (carfilzomib group) significantly improved progression-free survival (PFS) compared with lenalidomide and dexamethasone alone (control group) in patients with relapsed multiple myeloma (RMM). This post hoc a...

journal_title：British journal of haematology

authors： Dimopoulos MA,Stewart AK,Masszi T,Špička I,Oriol A,Hájek R,Rosiñol L,Siegel D,Mihaylov GG,Goranova-Marinova V,Rajnics P,Suvorov A,Niesvizky R,Jakubowiak A,San-Miguel J,Ludwig H,Palumbo A,Obreja M,Aggarwal S,Moreau P

更新日期：2017-05-01 00:00:00

abstract：:The object of this study was to determine whether there were any differences between the 'typical' child with fever and neutropenia and their adult counterpart with regard to infection type and outcome, by analysis of 3080 patients, including 759 children < 18 years of age and 2321 adults. These represented patients r...

journal_title：British journal of haematology

authors： Hann I,Viscoli C,Paesmans M,Gaya H,Glauser M

更新日期：1997-12-01 00:00:00

abstract：:Follicular lymphoma (FL) constitutes a significant proportion of lymphomas and shows frequent relapses. Beyond conventional chemotherapy, new therapeutic approaches have emerged, focussing on the interplay between lymphoma cells and the microenvironment. Here we report the immunophenotypic investigation of the microen...

journal_title：British journal of haematology

authors： Menter T,Tzankov A,Zucca E,Kimby E,Hultdin M,Sundström C,Beiske K,Cogliatti S,Banz Y,Cathomas G,Karjalainen-Lindsberg ML,Grobholz R,Mazzucchelli L,Sander B,Hawle H,Hayoz S,Dirnhofer S

更新日期：2020-05-01 00:00:00

abstract：:Novel therapies have transformed the treatment paradigm for multiple myeloma with significant improvements in survival now seen in both younger and older patients. Nonetheless, the disease is heterogeneous and high-risk patients in particular continue to have poor outcome. Moreover, the disease remains incurable. Effo...

journal_title：British journal of haematology

authors： Richardson PG,Laubach J,Mitsiades CS,Schlossman R,Hideshima T,Redman K,Chauhan D,Ghobrial IM,Munshi N,Anderson KC

更新日期：2011-09-01 00:00:00

abstract：:In patients with sickle cell anaemia (SCA), concomitant glucose-6-phosphate dehydrogenase (G6PD) deficiency is usually described as having no effect and only occasionally as increasing severity. We analysed sequential clinical and biological data for the first 42 months of life in SCA patients diagnosed by neonatal sc...

journal_title：British journal of haematology

authors： Benkerrou M,Alberti C,Couque N,Haouari Z,Ba A,Missud F,Boizeau P,Holvoet L,Ithier G,Elion J,Baruchel A,Ducrocq R

更新日期：2013-12-01 00:00:00

abstract：:The importance of the subclassification of nodular sclerosing Hodgkin's disease (NSHD) according to the British National Lymphoma Investigation Group (BNLI) criteria, which had an independent prognostic value for 90 unselected patients diagnosed in our region in the period 1972-83, is still equivocal. Because survival...

journal_title：British journal of haematology

authors： van Spronsen DJ,Vrints LW,Hofstra G,Crommelin MA,Coebergh JW,Breed WP

更新日期：1997-02-01 00:00:00

abstract：:A patient with pure red cell aplasia (PRCA) and autoimmune haemolytic anaemia (AIHA), associated with a thymoma which had already been removed, was studied in order to investigate the pathogenesis of PRCA and AIHA. The autoantibody eluted from the surface of the patient's red blood cells (RBC) reacted with the large E...

journal_title：British journal of haematology

authors： Taniguchi S,Shibuya T,Morioka E,Okamura T,Okamura S,Inaba S,Niho Y

更新日期：1988-04-01 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：:Thirty-four patients with refractory multiple myeloma were treated with 4-d continuous infusions of vincristine and adriamycin in combination with 4-d pulsed high-dose dexamethasone (VAD). Of 31 evaluable patients, 16 entered a complete remission (50%) and three a partial remission (10%). No difference in response rat...

journal_title：British journal of haematology

authors： Lokhorst HM,Meuwissen OJ,Bast EJ,Dekker AW

更新日期：1989-01-01 00:00:00

abstract：:Heparin-induced thrombocytopenia (HIT) is mediated by a heparin-dependent antibody/platelet factor 4/heparin complex binding to platelets via the Fc gamma receptor (type IIA). A single base polymorphism at position 131 of Fc gamma RIIA changes the native arginine to histidine. In the presence of murine monoclonal IgG1...

journal_title：British journal of haematology

authors： Burgess JK,Lindeman R,Chesterman CN,Chong BH

更新日期：1995-11-01 00:00:00

abstract：:The prognostic relevance of karyotype has been established in adult acute lymphoblastic leukaemia (ALL) patients treated with chemotherapy but not definitively evaluated in an allogeneic bone marrow transplantation (BMT) setting. To determine the factors affecting the outcome of allogeneic BMT for adults with precurso...

journal_title：British journal of haematology

authors： Lee S,Kim DW,Kim YJ,Park YH,Min CK,Lee JW,Min WS,Kim CC

更新日期：2002-04-01 00:00:00