The cytoskeletal linker protein moesin: decreased levels in Wiskott-Aldrich syndrome platelets and identification of a cleavage pathway in normal platelets.

abstract：:Murine monoclonal antibodies (MoAbs) were produced against the blood group KEL1 glycoprotein (93 kD component) immunopurified from human erythrocytes. One monoclonal antibody, 5A11 (IgGa, kappa), detects by immunoblotting a 93 and 184 kD component from KEL: 1,-2 or KEL: -1,2 red cell membrane preparations, separated b...

journal_title：British journal of haematology

authors： Jaber A,Loirat MJ,Willem C,Bloy C,Cartron JP,Blanchard D

更新日期：1991-10-01 00:00:00

abstract：:The routine methods for estimating erythrocyte size (volume and surface area) are not generally unbiased. Using geometric models introduces a bias determined by the ways in which real red blood corpuscles depart in size and shape from the simplistic ideal. Employing haematocrit and red corpuscle count to estimate mean...

journal_title：British journal of haematology

authors： Mayhew TM,Mwamengele GL,Self TJ,Travers JP

更新日期：1994-02-01 00:00:00

abstract：:Heparin binding on polymorphonuclear leucocytes (PMNL) was characterized. Heparin binding was specific, rapid, saturable and reversible. One single class of heparin binding sites was found with a dissociation constant of 1.22 mumol/l and 7.7 x 10(6) sites per PMNL. The binding was independent of the anticoagulant acti...

journal_title：British journal of haematology

authors： Leculier C,Benzerara O,Couprie N,Francina A,Lasne Y,Archimbaud E,Fiere D

更新日期：1992-05-01 00:00:00

abstract：:To evaluate the clinical significance of CD21S expression of diffuse large B-cell lymphoma (DLBCL) tumour cells, we compared their clinical features, immunophenotype, response to therapy and outcome in relation to CD21S expression. Between 1987 and 1999, frozen sections from 240 DLBCL cases were examined for CD21S exp...

journal_title：British journal of haematology

authors： Ogawa S,Yamaguchi M,Oka K,Taniguchi M,Ito M,Nishii K,Nakase K,Ohno T,Kita K,Kobayashi T,Shiku H

更新日期：2004-04-01 00:00:00

abstract：:Chromosomal rearrangements involving the MECOM (MDS1 and EVI1 complex) locus are recurrent genetic events in myeloid leukaemia and are associated with poor prognosis. In this study, we assessed the role of MECOM locus protein EVI1 in the transcriptional regulation of microRNAs (miRNAs) involved in the leukaemic phenot...

journal_title：British journal of haematology

authors： De Weer A,Van der Meulen J,Rondou P,Taghon T,Konrad TA,De Preter K,Mestdagh P,Van Maerken T,Van Roy N,Jeison M,Yaniv I,Cauwelier B,Noens L,Poirel HA,Vandenberghe P,Lambert F,De Paepe A,Sánchez MG,Odero M,Verhasselt

更新日期：2011-08-01 00:00:00

abstract：:Sickle cell disease (SCD) is complicated by silent cerebral infarcts, visible as white matter hyperintensities (WMHs) on magnetic resonance imaging (MRI). Both local vaso-occlusion, elicited by endothelial dysfunction, and insufficiency of cerebral blood flow (CBF) have been proposed to be involved in the aetiology. W...

journal_title：British journal of haematology

authors： van der Land V,Mutsaerts HJ,Engelen M,Heijboer H,Roest M,Hollestelle MJ,Kuijpers TW,Nederkoorn PJ,Cnossen MH,Majoie CB,Nederveen AJ,Fijnvandraat K

更新日期：2016-01-01 00:00:00

abstract：:In two unrelated Spanish males with glucose-6-phosphate dehydrogenase (G6PD) deficiency and haemolytic anaemia, and two different novel point mutations in the G6PD gene, have been identified. A C to T transition at nucleotide 406 resulting in a (136) Arg to Cys substitution and a C to G transition at nucleotide 1155 r...

journal_title：British journal of haematology

authors： Zarza R,Pujades A,Rovira A,Saavedra R,Fernandez J,Aymerich M,Vives Corrons JL

更新日期：1997-09-01 00:00:00

abstract：:Hereditary angioedema (HAE) is a rare autosomal dominant genetic disorder clinically characterized by recurrent attacks of subcutaneous and mucosal swelling that can result in significant morbidity and even mortality. Several novel therapies introduced since 2008 have dramatically transformed the approach to managemen...

journal_title：British journal of haematology

authors： Zuraw BL,Christiansen SC

更新日期：2016-06-01 00:00:00

abstract：:The feasibility of marrow cryopreservation for autologous bone marrow transplantation after 7 d in liquid culture was assessed in 10 leukaemic patients. A median of 0.17 x 10(8) nucleated cells/kg and 0.4 x 10(4) CFU-GM/kg could be collected after the complete procedure, with overall a consistent cell loss. Long-term ...

journal_title：British journal of haematology

authors： Stoppa AM,Maraninchi D,Lafage M,Mannoni P,Novakovitch G,Blaise D,Gaspard MH,Michel G,Carcassonne Y

更新日期：1989-08-01 00:00:00

abstract：:Endothelial progenitor cells (EPCs) were recently demonstrated to exist in human cord blood. Phytohaemagglutinin (PHA), a potent mitogen for mononuclear cells was used to induce EPCs from unsorted cord blood mononuclear cells (CBMCs). Adherent cells in clusters appeared approximately 24 h after CBMCs were cultured in ...

journal_title：British journal of haematology

authors： Kang HJ,Kim SC,Kim YJ,Kim CW,Kim JG,Ahn HS,Park SI,Jung MH,Choi BC,Kimm K

更新日期：2001-06-01 00:00:00

abstract：:The outcome of children with acute lymphoblastic leukaemia (ALL) and early relapse remains unsatisfactory. In January 1995, the AIEOP (Associazione Italiana di Oncologia ed Ematologia Pediatrica) group opened a trial for children with ALL in first isolated or combined bone marrow relapse defined at high risk according...

journal_title：British journal of haematology

authors： Testi AM,Del Giudice I,Arcese W,Moleti ML,Giona F,Basso G,Biondi A,Conter V,Messina C,Rondelli R,Micozzi A,Micalizzi C,Barisone E,Locatelli F,Dini G,Aricò M,Casale F,Comis M,Ladogana S,Lippi A,Mura R,Pinta MF,

更新日期：2002-09-01 00:00:00

abstract：:In 2019 the UK Myeloma Research Alliance introduced the Myeloma Risk Profile (MRP) for prediction of outcome in patients with newly diagnosed multiple myeloma (MM), ineligible for autologous stem cell transplantation. To validate the MRP in a population-based setting we performed a study of the entire cohort of transp...

journal_title：British journal of haematology

authors： Redder L,Klausen TW,Vangsted AJ,Gregersen H,Andersen NF,Pedersen RS,Szabo AG,Frederiksen M,Frølund UC,Helleberg C,Nielsen LK,Pedersen PT,Salomo M,Gimsing P,Frederiksen H,Abildgaard N

更新日期：2020-06-09 00:00:00

abstract：:Decreased bone formation plays an important role in the development of lytic lesions during the late stage of multiple myeloma (MM). Release of insulin-like growth factor binding protein-4 (IGFBP4) by tumour cells adjacent to bone may inhibit IGF-I-stimulated osteoblast growth and contribute to decreased bone formatio...

journal_title：British journal of haematology

authors： Feliers D,Woodruff K,Abboud S

更新日期：1999-03-01 00:00:00

abstract：:Massive genomic analyses have underscored the diversity of chronic lymphocytic leukaemia (CLL) between patients. Genetic heterogeneity of tumour clones within a patient may fuel tumour evolution. Several recurrently deregulated intra-cellular pathways are candidates for targeted therapies that are very promising and a...

journal_title：British journal of haematology

authors： Ghamlouch H,Nguyen-Khac F,Bernard OA

更新日期：2017-09-01 00:00:00

abstract：:A 41-year-old woman was admitted with fever, splenomegaly and pancytopenia. High serum ferritin, hypertriglyceridaemia and bone marrow haemophagocytosis were consistent with a haemophagocytic syndrome. Trophozoites and gametocytes of Plasmodium vivax were identified on blood smear. Rapid recovery was observed after tr...

journal_title：British journal of haematology

authors： Aouba A,Noguera ME,Clauvel JP,Quint L

更新日期：2000-03-01 00:00:00

abstract：:A patient presented with haemolytic anaemia and a negative direct antiglobulin test (DAT), and was found to have an IgG antibody with anti-Jka specificity in his serum. His red cells were typed as Jk(a-b+). Later he developed idiopathic thrombocytopenic purpura (ITP), and had a positive DAT due to anti-Jka bound to hi...

journal_title：British journal of haematology

authors： Ganly PS,Laffan MA,Owen I,Hows JM

更新日期：1988-08-01 00:00:00

abstract：:Management of the acute painful crisis (APC) of sickle cell disease (SCD) remains unsatisfactory despite advances in the understanding and management of acute pain in other clinical settings. One reason for this is an unsophisticated approach to the use of opioid analgesics for pain management. This applies to haemato...

journal_title：British journal of haematology

authors： Telfer P,Bahal N,Lo A,Challands J

更新日期：2014-07-01 00:00:00

abstract：:The transcriptome of the CD34+ cells was determined in a group of 10 patients with the 5q- syndrome using a comprehensive array platform, and was compared with the transcriptome of CD34+ cells from 16 healthy control subjects and 14 patients with refractory anaemia and a normal karyotype. The majority of the genes ass...

journal_title：British journal of haematology

authors： Boultwood J,Pellagatti A,Cattan H,Lawrie CH,Giagounidis A,Malcovati L,Della Porta MG,Jädersten M,Killick S,Fidler C,Cazzola M,Hellström-Lindberg E,Wainscoat JS

更新日期：2007-11-01 00:00:00

abstract：:Hydroxycarbamide (HC) (or hydroxyurea) has been reported to increase fetal haemoglobin levels and improve clinical symptoms in sickle cell anaemia (SCA) patients. However, the complete pathway by which HC acts remains unclear. To study the mechanisms involved in the action of HC, global gene expression profiles were o...

journal_title：British journal of haematology

authors： Costa FC,da Cunha AF,Fattori A,de Sousa Peres T,Costa GG,Machado TF,de Albuquerque DM,Gambero S,Lanaro C,Saad ST,Costa FF

更新日期：2007-01-01 00:00:00

abstract：:In this population-based survey covering two geographically distinct UK regions, we evaluated the number of myeloma patients aged < or =65 years who have not undergone transplantation. The combined data from both of these regions showed that 57% of age-eligible patients were not transplanted. While early death and com...

journal_title：British journal of haematology

authors： Morris TC,Velangi M,Jackson G,Marks DI,Ranaghan L,Northern Ireland Regional Haematology Group.,Northern Regional Haematologists Group.,Clinical Trials Committee of The British Scoiety for Blood and Marrow Transplantation.

更新日期：2005-02-01 00:00:00

abstract：:The combination of bortezomib and rituximab was evaluated in patients with mantle cell lymphoma (MCL), follicular lymphoma (FL) and Waldenström macroglobulinaemia (WM), in a Phase I and later, a randomized Phase II study. In the randomized study, 42 patients with recurrent/refractory disease received either: bortezomi...

journal_title：British journal of haematology

authors： Agathocleous A,Rohatiner A,Rule S,Hunter H,Kerr JP,Neeson SM,Matthews J,Strauss S,Montoto S,Johnson P,Radford J,Lister A

更新日期：2010-11-01 00:00:00

abstract：:The odds ratio of homozygosity or heterozygosity for the TNF2 polymorphism in 575 patients with venous thromboembolism compared to controls was found to be 1.0 (95% CI 0.4-2.1) and 1.1 (95% CI 0.8-1.4) respectively. Comparing subgroups of patients and controls with the factor V Leiden mutation the odds ratio for the T...

journal_title：British journal of haematology

authors： Brown K,Luddington R,Baglin T

更新日期：1998-06-01 00:00:00

abstract：:Changes in DNA copy number (CN) and DNA methylation represent important aberrations for lymphomas and other cancers. Here, for the first time, we show that the Illumina Infinium Methylation (IIM) assay, although not originally designed for CN profiling, is able to estimate CN changes. We compared the IIM CN profiles t...

journal_title：British journal of haematology

authors： Kwee I,Rinaldi A,Rancoita P,Rossi D,Capello D,Forconi F,Giuliani N,Piva R,Inghirami G,Gaidano G,Zucca E,Bertoni F

更新日期：2012-02-01 00:00:00

abstract：:A patient with familial analpha-lipoproteinaemia (Tangier disease) was found to have stomatocytosis and haemolytic anaemia. The analysis of the red cell membrane constituents revealed a low cholesterol content (90 nmol/ml red cells, control 130 nmol/ml red cells), a decreased cholesterol/phospholipid ratio (0.54, cont...

journal_title：British journal of haematology

authors： Reinhart WH,Gössi U,Bütikofer P,Ott P,Sigrist H,Schatzmann HJ,Lutz HU,Straub PW

更新日期：1989-06-01 00:00:00

abstract：:Familial haemophagocytic lymphohistiocytosis (FHLH) is a genetic disorder caused by defective lymphocyte cytotoxicity, resulting in impaired lymphocyte homeostasis and macrophage infiltration of solid tissues and bone marrow, with extensive haemophagocytosis. It is invariably fatal unless treated by allogeneic haemato...

journal_title：British journal of haematology

authors： Almousa H,Ouachée-Chardin M,Picard C,Radford-Weiss I,Caillat-Zucman S,Cavazzana-Calvo M,Blanche S,de Saint Basile G,Le Deist F,Fischer A

更新日期：2005-08-01 00:00:00

abstract：:This study aimed to assess the potential of human cord blood (CB) cells to engraft in the xenogenic non-obese diabetic/severe combined immunodeficient (NOD/SCID) mouse model after in vitro expansion culture. We also studied the quality of human haemopoiesis arising from the transplantation of fresh or expanded cells i...

journal_title：British journal of haematology

authors： Denning-Kendall PA,Evely R,Singha S,Chapman M,Bradley BA,Hows JM

更新日期：2002-01-01 00:00:00

abstract：:We attempted to identify the diagnostic markers and severity predictors of hepatic veno-occlusive disease (VOD) in 115 adult patients who were uniformly conditioned with busulphan and cyclophosphamide and who underwent allogeneic bone marrow transplantation (BMT). A diagnosis of VOD was made according to clinical crit...

journal_title：British journal of haematology

authors： Lee JH,Lee KH,Lee JH,Kim S,Seol M,Park CJ,Chi HS,Kang W,Kim ST,Kim WK,Lee JS

更新日期：2002-09-01 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：:We observed macrothrombocytopenia with leucocyte inclusions in 10 out of 14 members of a four-generation family. Morphological features of leucocyte inclusions and the presence of Alport-related symptoms supported the diagnosis of Fechtner syndrome. Compared to the two previously reported Fechtner families, our kindre...

journal_title：British journal of haematology

authors： Rocca B,Laghi F,Zini G,Maggiano N,Landolfi R

更新日期：1993-10-01 00:00:00

abstract：:Two single-nucleotide substitutions in PKLR constituted the molecular basis underlying pyruvate kinase (PK) deficiency in a patient with severe haemolytic anaemia. One novel mutation, IVS5+1G>A, abolished the intron 5 donor splice site. The other mutation, c.1436G>A, altered the intron 10 donor splice site consensus s...

journal_title：British journal of haematology

authors： Wijk R,van Wesel AC,Thomas AA,Rijksen G,van Solinge WW

更新日期：2004-04-01 00:00:00