New genotypes in Fy(a-b-) individuals: nonsense mutations (Trp to stop) in the coding sequence of either FY A or FY B.

abstract：:The interaction of rare Hb variants with beta(0)-thalassaemia results in a quasihomozygous state where the erythrocytes contain the variant as the only major adult Hb component. Such a situation is a unique model that enables functional studies even in the case of a neutral variant that could not be isolated from Hb A...

journal_title：British journal of haematology

authors： Vassilopoulos G,Papassotiriou I,Voskaridou E,Stamoulakatou A,Premetis E,Kister J,Marden M,Griffon N,Poyart C,Wajcman H

更新日期：1995-11-01 00:00:00

abstract：:The first step in thrombin-induced aggregation of blood platelets is binding of thrombin to specific receptors on the platelet membrane. Elucidation of the nature of this receptor in human platelets was attempted using radioactively labelled thrombin. In disc gel electrophoresis an extract of thrombin-treated platelet...

journal_title：British journal of haematology

authors： Ganguly P

更新日期：1975-04-01 00:00:00

abstract：:Leucocyte interferon production in vitro and circulating interferon levels were studied in healthy subjects and in 80 patients with acute or chronic leukaemia. Circulating interferon was not found in either group. Interferon synthesis in response to a virus was normal in patients with acute leukaemia and appeared to b...

journal_title：British journal of haematology

authors： Chisholm M,Cartwright T

更新日期：1978-09-01 00:00:00

abstract：:It has been proposed that when ABO unmatched platelets are transfused circulating immune complexes (CIC) may be formed between the patient's soluble ABH antigens and the transfused antibodies. Platelets might then be destroyed by bystander mechanisms or by the binding of CIC to the Fc receptor or to C3 binding membran...

journal_title：British journal of haematology

authors： Heal JM,Masel D,Rowe JM,Blumberg N

更新日期：1993-11-01 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：:Sézary syndrome and Mycosis fungoides are the most common forms of cutaneous T-cell lymphomas. To assess the response to different therapies especially in Sézary syndrome, it is helpful to monitor the percentage of circulating tumour cells in the blood. The use of T-cell receptor (TCR)-Vbeta specific monoclonal antibo...

journal_title：British journal of haematology

authors： Schwab C,Willers J,Niederer E,Ludwig E,Kündig T,Grob P,Burg G,Dummer R

更新日期：2002-09-01 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：:Using two-colour phenotypic analysis with anti-CD38 antibody, human myeloma cells can be classified into VLA-5- immature and VLA-5+ mature cells. We examined the relationship between variations of these subpopulations and clinical responses during treatment in multiple myeloma (MM). 39 patients with MM were treated wi...

journal_title：British journal of haematology

authors： Kawano MM,Mahmoud MS,Huang N,Lisukov IA,Mihara K,Tsujimoto T,Kuramoto A

更新日期：1995-12-01 00:00:00

abstract：:Clofarabine was the latest new drug to be approved, in 2004, for relapsed or refractory acute lymphoblastic leukaemia (ALL). To investigate its value in the frontline treatment of ALL we applied clofarabine 5 × 40 mg/m(2) in combination with pegylated asparaginase (PEG-ASP) 1 × 2500 iu/m(2) in high risk ALL patients a...

journal_title：British journal of haematology

authors： Escherich G,Zur Stadt U,Zimmermann M,Horstmann MA,CoALL study group.

更新日期：2013-10-01 00:00:00

abstract：:We investigated the molecular basis of factor VII deficiency in a Japanese patient and identified a novel missense mutation in the signal sequence of the gene. Factor VII activity and antigen level measured in the patient were 10.7% and 11% of normal, respectively. All exons except 1B and the 5'-flanking region contai...

journal_title：British journal of haematology

authors： Ozawa T,Takikawa Y,Niiya K,Ejiri N,Suzuki K,Sato S,Sakuragawa N

更新日期：1998-04-01 00:00:00

abstract：:We describe the characterization of a novel 7.9 kb deletion that eliminated one of the duplicated alpha-globin genes, causing an alpha+-thalassaemia phenotype in two independent carriers of Suriname-Indian origin. The molecular characterization of the deletion breakpoint fragment revealed neither involvement of Alu re...

journal_title：British journal of haematology

authors： Harteveld CL,van Delft P,Wijermans PW,Kappers-Klunne MC,Weegenaar J,Losekoot M,Giordano PC

更新日期：2003-01-01 00:00:00

abstract：:To examine any role of macrophage colony-stimulating factor (M-CSF), in the immune responses in Kawasaki disease (KD), we serially assayed M-CSF and several related cytokines using ELISA. In 10 paediatric patients with KD the level of M-CSF was significantly higher in the acute phase than in the convalescent phase (14...

journal_title：British journal of haematology

authors： Igarashi H,Hatake K,Tomizuka H,Yamada M,Gunji Y,Momoi MY

更新日期：1999-06-01 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：:A retrospective analysis of CD20 expression following rituximab for B-cell non-Hodgkin's lymphoma demonstrated a significant change in immunophenotype in 6/25 (24%) patients with persistent bone marrow (BM) infiltration. In three out of six patients, the B cells were uniformly CD20-/CD79alpha+, consistent with frank l...

journal_title：British journal of haematology

authors： Foran JM,Norton AJ,Micallef IN,Taussig DC,Amess JA,Rohatiner AZ,Lister TA

更新日期：2001-09-01 00:00:00

abstract：:Data on outcome, prognostic factors, and treatment for very elderly non-Hodgkin lymphomas (NHL) is sparse. We conducted a multicentre retrospective analysis of NHL patients ≥80 years (at diagnosis) treated between 1999 and 2009. Detailed characteristics were obtained including geriatric syndromes, activities of daily ...

journal_title：British journal of haematology

authors： Nabhan C,Smith SM,Helenowski I,Ramsdale E,Parsons B,Karmali R,Feliciano J,Hanson B,Smith S,McKoy J,Larsen A,Hantel A,Gregory S,Evens AM

更新日期：2012-01-01 00:00:00

abstract：:Erythropoietin (EPO) and stem cell factor (SCF) are two important factors in human erythropoiesis. We have recently demonstrated that SCF and EPO synergistically activate mitogen-activated protein (MAP) kinase, thereby promoting growth of human erythroid colony-forming cells (ECFCs). In the present study, we have exam...

journal_title：British journal of haematology

authors： Sui X,Krantz SB,Zhao ZJ

更新日期：2000-07-01 00:00:00

abstract：:To investigate the role of neutrophils in complement-induced changes in vascular permeability, skin wheal and flare responses to intradermal injection of autologous activated serum complement were measured in normal and neutropenic subjects. In normal subjects, responses were dose-dependent and were abolished by remov...

journal_title：British journal of haematology

authors： Williamson LM,Sheppard K,Davies JM,Fletcher J

更新日期：1986-10-01 00:00:00

abstract：:Recent findings have indicated the importance of factor V (FV) in causing resistance to activated protein C (APC) in a high proportion of patients with venous thrombosis. This prompted us to investigate whether resistance could be due to defective inactivation of FVa by APC. Consequently, we amplified a 3.2 kb fragmen...

journal_title：British journal of haematology

authors： Beauchamp NJ,Daly ME,Hampton KK,Cooper PC,Preston FE,Peake IR

更新日期：1994-09-01 00:00:00

abstract：:Platelets in an infant with Chediak-Higashi (C-H) syndrome without bleeding manifestations and not in the accelerated phase showed abnormal function consistent with storage pool disorder as shown by abnormal aggregation, decreased storage capacity and release of [14C]5-HT, low endogenous 5-HT, reduced ATP and ADP with...

journal_title：British journal of haematology

authors： Boxer GJ,Holmsen H,Robkin L,Bang NU,Boxer LA,Baehner RL

更新日期：1977-04-01 00:00:00

abstract：:Human IgG monoclonal antibodies (mabs) against the Rh D antigen have considerable potential for the prophylaxis of haemolytic disease of the newborn. We have carried out in vitro testing for cross-reactions with tissue components by screening two such mabs against animal tissues and a wide panel of human organs from n...

journal_title：British journal of haematology

authors： Thorpe SJ,Bailey SW

更新日期：1993-02-01 00:00:00

abstract：:Over the past four decades, remarkable progress has been made in the treatment and prognosis of multiple myeloma (MM), although it remains an incurable disease. Chemotherapy resistance is a major hurdle for treatment efficacy. Drug resistance can be innate and so driven by genes involved in the drug metabolism pathway...

journal_title：British journal of haematology

authors： Macauda A,Castelli E,Buda G,Pelosini M,Butrym A,Watek M,Kruszewski M,Vangsted AJ,Rymko M,Jamroziak K,Abildgaard N,Haastrup EK,Mazur G,Ríos R,Jurczyszyn A,Zawirska D,Dudziński M,Raźny M,Dutka M,Tomczak W,Suska A,

更新日期：2018-11-01 00:00:00

abstract：:Langerhans cell histiocytosis (LCH), the most common histiocytic disorder, is characterized by the accumulation of CD1A(+) /CD207(+) mononuclear phagocytes within granulomatous lesions that can affect nearly all organ systems. Historically, LCH has been presumed to arise from transformed or pathologically activated ep...

journal_title：British journal of haematology

authors： Berres ML,Merad M,Allen CE

更新日期：2015-04-01 00:00:00

abstract：:Previous studies have shown that interleukin 2 (IL2) production by peripheral blood mononuclear cells (PBMC) is severely impaired post allogeneic bone marrow transplantation, whereas production of interferon-gamma (IFN-gamma) is at most marginally depressed. To investigate the mechanisms behind this apparently differe...

journal_title：British journal of haematology

authors： Cooley MA,McLachlan K,Atkinson K

更新日期：1989-11-01 00:00:00

abstract：:The activities of glucose-6-phosphate dehydrogenase (D-glucose-6-phosphate: NADP oxidoreductase, G6PD), 6-phosphogluconate dehydrogenase (6-phospho-D-gluconate: NADP oxidoreductase, 6PGD), hexokinase (ATP: D-hexose 6-phosphotransferase, Hx), lactate dehydrogenase (D-lactate: NAD oxidoreductase, LDH). glutamate oxaloac...

journal_title：British journal of haematology

authors： Brok-Simoni F,Ashkenazi YE,Holtzman F,Ramot B

更新日期：1975-02-01 00:00:00

abstract：:Summary Previous findings of megakaryocytic hypogranulation and dysmegakaryocytopoietic features in acute myeloid leukaemia (AML) strongly indicate defects in platelet production. The bleeding tendency of these patients may result from dysregulated platelet production, resulting in thrombocytopenia as well as qualitat...

journal_title：British journal of haematology

authors： Leinoe EB,Hoffmann MH,Kjaersgaard E,Johnsen HE

更新日期：2004-10-01 00:00:00

abstract：:A simplified method of International Normalized Ratio (INR) derivation using linear regression of certified INR plotted against local prothrombin time (PT) results has been compared with INR from conventional orthogonal regression. Linear regression assumes error only with the local PT results whereas orthogonal regre...

journal_title：British journal of haematology

authors： Poller L,Barrowcliffe TW,van den Besselaar AM,Jespersen J,Tripodi A,Houghton D

更新日期：1997-09-01 00:00:00

abstract：:A woman with Ph-positive chronic myeloid leukaemia (CML) with an atypical e1a3 BCR-ABL hybrid gene is described. To our knowledge, this is the first report of this transcript type as a unique naturally occurring BCR-ABL fusion in a CML patient. This case was characterized by a low leucocyte count and a very indolent c...

journal_title：British journal of haematology

authors： Roman J,Jimenez A,Barrios M,Castillejo JA,Maldonado J,Torres A

更新日期：2001-09-01 00:00:00

abstract：:To assess and define the effects of heterocellular hereditary persistence of fetal haemoglobin (HPFH) on the haematological phenotype of heterozygous beta-thalassaemia, we have studied a large kindred that included a total of 204 subjects with 60 beta-thalassaemia carriers, of whom 35 were also heterozygous, and five ...

journal_title：British journal of haematology

authors： Garner C,Dew TK,Sherwood R,Rees D,Thein SL

更新日期：2003-10-01 00:00:00

abstract：:Severe neutropenia and protracted thrombocytopenia remain serious clinical problems following cord blood transplantation (CBT) due to the paucity of stem and progenitor cells in the grafts. Administration of ex-vivo expanded megakaryocyte progenitor cells may facilitate platelet production. We propose a novel strategy...

journal_title：British journal of haematology

authors： Deutsch V,Hubel E,Kay S,Ohayon T,Katz BZ,Many A,Zander A,Naparstek E,Grisaru D

更新日期：2010-04-01 00:00:00

abstract：:We report the characterization of three variant antithrombins with reduced heparin binding as the primary abnormality. Two of these variants, antithrombin Southport (Leu 99 to Val, 2759 C to G) and antithrombin Vienna (Gln 118 to Pro, 5349 A to C) were novel, whereas the third, Pro 41 to Leu, has been previously descr...

journal_title：British journal of haematology

authors： Chowdhury V,Mille B,Olds RJ,Lane DA,Watton J,Barrowcliffe TW,Pabinger I,Woodcock BE,Thein SL

更新日期：1995-03-01 00:00:00