Characterization of two novel splice site mutations in human factor VII gene causing severe plasma factor VII deficiency and bleeding diathesis.

abstract：:Phenotypic change of blood-type eosinophils to tissue-type eosinophils is induced by various cytokines. We examined the effect of nerve growth factor (NGF) as a candidate for a constitutive cytokine which is able to induce the phenotypic change of eosinophils. The viability of human peripheral blood eosinophils cultur...

journal_title：British journal of haematology

authors： Hamada A,Watanabe N,Ohtomo H,Matsuda H

更新日期：1996-05-01 00:00:00

abstract：:In vitro culture of hairy cells (HC) (five patients) with alpha IFN (100 U/ml) significantly enhanced MHC and CD22 antigen expression and reduced CD25, sIg and FMC7 positivity, together with consistent but not significant reductions in CD9, 19 and HC2. A sixth patient, who was refractory to the effects of alpha IFN in...

journal_title：British journal of haematology

authors： Till KJ,Cawley JC

更新日期：1989-07-01 00:00:00

abstract：:Isolated trisomy 8 (+8) is a frequent cytogenetic abnormality in the myelodysplastic syndromes (MDS), but its characteristics are poorly reported. We performed a retrospective study of 138 MDS patients with isolated +8, classified or reclassified as MDS (excluding MDS/myeloproliferative neoplasm). Myeloproliferative (...

journal_title：British journal of haematology

authors： Drevon L,Marceau A,Maarek O,Cuccuini W,Clappier E,Eclache V,Cluzeau T,Richez V,Berkaoui I,Dimicoli-Salazar S,Bidet A,Vial JP,Park S,Vieira Dos Santos C,Kaphan E,Berthon C,Stamatoullas A,Delhommeau F,Abermil N,Braun

更新日期：2018-09-01 00:00:00

abstract：:Since glycoprotein IV (GPIV) has been shown to play an important role in the interaction of platelets with collagen and thrombospondin, the aggregation and secretion of GPIV-deficient platelets were examined. Using a binding assay with monoclonal 125I-OKM5 antibody against CD36 antigen and crossed immunoelectrophoresi...

journal_title：British journal of haematology

authors： Yamamoto N,Akamatsu N,Yamazaki H,Tanoue K

更新日期：1992-05-01 00:00:00

abstract：:Beta2-glycoprotein I (beta2GPI) is an important target antigen for antiphospholipid antibodies (aPL) and thus beta2GPI polymorphisms may influence aPL production and the development of antiphospholipid syndrome. We have studied the relationship between the Val247Leu and Trp316Ser beta2GPI polymorphisms and the aPL sta...

journal_title：British journal of haematology

authors： Camilleri RS,Mackie IJ,Humphries SE,Machin SJ,Cohen H

更新日期：2003-03-01 00:00:00

abstract：:Ex vivo expansion of primitive human haematopoietic stem cells (HSC) is clinically relevant for stem cell transplantation and gene therapy. Here, we demonstrate the selective expansion of CD34+CD38- cells from purified CD34+ cells upon stimulation with Flt3-ligand, stem cell factor and thrombopoietin. Over a 100-fold ...

journal_title：British journal of haematology

authors： Ng YY,Bloem AC,van Kessel B,Lokhorst H,Logtenberg T,Staal FJ

更新日期：2002-04-01 00:00:00

abstract：:Standard treatment for classical Hodgkin lymphoma (cHL) is poorly tolerated in older patients and results disappointing. We assessed safety and efficacy of brentuximab vedotin (BV), in previously untreated patients with cHL unfit for standard treatment due to age, frailty or comorbidity. The primary outcome was comple...

journal_title：British journal of haematology

authors： Gibb A,Pirrie SJ,Linton K,Warbey V,Paterson K,Davies AJ,Collins GP,Menne T,McKay P,Fields PA,Miall FM,Nagy E,Wheatley K,Reed R,Baricevic-Jones I,Barrington S,Radford J

更新日期：2020-09-14 00:00:00

abstract：:A t(5;12)(q33;p13) translocation has been detected in two patients with myeloid disorder and eosinophilia. Six other patients with haematological disease with eosinophilia with similar translocation have been published previously. The existence of a new entity, a myeloproliferative disorder with eosinophilia and t(5;1...

journal_title：British journal of haematology

authors： Baranger L,Szapiro N,Gardais J,Hillion J,Derre J,Francois S,Blanchet O,Boasson M,Berger R

更新日期：1994-10-01 00:00:00

abstract：:The clinical characteristics and prognostic relevance of acute myeloid leukaemia (AML) with myelodysplastic features remains to be clarified in children. We prospectively examined 443 newly diagnosed patients in a multicentre clinical trial for paediatric de novo AML, and found 'AML with myelodysplasia-related changes...

journal_title：British journal of haematology

authors： Kinoshita A,Miyachi H,Matsushita H,Yabe M,Taki T,Watanabe T,Saito AM,Tomizawa D,Taga T,Takahashi H,Matsuo H,Kodama K,Ohki K,Hayashi Y,Tawa A,Horibe K,Adachi S

更新日期：2014-10-01 00:00:00

abstract：:The existence of a phenomenon of rebound hypercoagulability after cessation of oral anticoagulant therapy is controversial. The sensitive procoagulant markers for in vivo thrombin and fibrin formation are potential tools for the reassessment of the presence of each a phenomenon. We examined 19 patients anticoagulated ...

journal_title：British journal of haematology

authors： Genewein U,Haeberli A,Straub PW,Beer JH

更新日期：1996-02-01 00:00:00

abstract：:An indirect immunofluorescence technique with single and double labelling has been used to examine cultured human megakaryocytes for ABH antigens. This technique demonstrated the presence of these antigens on megakaryocytes and a population of small mononuclear cells that probably represent the differentiated precurso...

journal_title：British journal of haematology

authors： Dunstan RA

更新日期：1986-03-01 00:00:00

abstract：:We report on quantitative analyses of C5b-9 binding to target red blood cells (RBC) lysed through the action of drug (nomifensine)-dependent antibodies (ddab) and anti-RBC antibodies (warm plus cold agglutinins). Immunoradiometric assays showed that, at any given degree of haemolysis, more C5b-9 was bound to cells sen...

journal_title：British journal of haematology

authors： Salama A,Mueller-Eckhardt C,Boschek B,Bhakdi S

更新日期：1987-02-01 00:00:00

abstract：:Two children affected by severe aplastic anaemia (SAA) underwent allogeneic bone marrow transplantation (BMT) using partially matched family donors. In both cases there was a successful engraftment of donor haemopoietic stem cells. However, after an initial erythropoietic recovery, 5 months following BMT both children...

journal_title：British journal of haematology

authors： Locatelli F,Pedrazzoli P,Barosi G,Zecca M,Porta F,Liberato L,Gambarana D,Nespoli L,Cazzola M

更新日期：1992-04-01 00:00:00

abstract：:Polycythaemia vera (PV) patients' blood burst-forming units-erythroid (BFU-E) have an enhanced sensitivity to stem cell factor (SCF) compared to normal BFU-E. To characterize SCF receptors on erythroid progenitors from normal individuals and PV patients, we performed binding experiments using radioiodinated recombinan...

journal_title：British journal of haematology

authors： Dai CH,Krantz SB,Koury ST,Kollar K

更新日期：1994-11-01 00:00:00

abstract：:Routine testing on plasma from a patient due to undergo a coronary artery bypass graft operation revealed a prolonged thrombin clotting time associated with a normal plasma fibrinogen level when this was determined by a method not dependent upon the rate of fibrin formation. Fibrinogen purified from the patient's plas...

journal_title：British journal of haematology

authors： Lane DA,Cuddigan B,VanRoss M,Kakkar VV

更新日期：1980-03-01 00:00:00

abstract：:Excessive bleeding represents a major complication of surgical interventions and its control is especially relevant in patients with Congenital Bleeding Disorders (CBD). In factor VII (FVII) deficiency, scanty data on surgery is available to guide treatment strategies. The STER (Seven Treatment Evaluation Registry) is...

journal_title：British journal of haematology

authors： Mariani G,Dolce A,Batorova A,Auerswald G,Schved JF,Siragusa S,Napolitano M,Knudsen JB,Ingerslev J,STER and the International Factor VII Deficiency Study Groups.

更新日期：2011-02-01 00:00:00

abstract：:To study the pathogenesis of multicentric Castleman's disease (MCD), IL-6 producing cells and immune function were investigated in four MCD patients. The expression of IL-6 mRNA in one MCD lymph node was analysed by in situ hybridization. IL-6 mRNA expressing cells were scattered in the interfollicular areas and did n...

journal_title：British journal of haematology

authors： Ishiyama T,Nakamura S,Akimoto Y,Koike M,Tomoyasu S,Tsuruoka N,Murata Y,Sato T,Wakabayashi Y,Chiba S

更新日期：1994-03-01 00:00:00

abstract：:Comparative expression analysis of wild-typeETV6 in the disease state showed an absence of expression in ETV6-CBFA2 acute lymphoblastic leukaemia (ALL) when compared with non-ETV6-CBFA2 ALL and acute myeloid leukaemia. Fluorescent in-situ hybridization and loss of heterozygosity studies showed that 73% of the ETV6-CBF...

journal_title：British journal of haematology

authors： Patel N,Goff LK,Clark T,Ford AM,Foot N,Lillington D,Hing S,Pritchard-Jones K,Jones LK,Saha V

更新日期：2003-07-01 00:00:00

abstract：:Dyskeratosis congenita (DC) is an inherited disorder characterized by skin pigmentation, nail dystrophy and mucosal leucoplakia. In 1995 a Dyskeratosis Congenita Registry was established at the Hammersmith Hospital. In the 46 families recruited, 76/83 patients were male, suggesting that the major form of DC is X-linke...

journal_title：British journal of haematology

authors： Knight S,Vulliamy T,Copplestone A,Gluckman E,Mason P,Dokal I

更新日期：1998-12-01 00:00:00

abstract：:Mantle-cell lymphoma (MCL) is a B-cell tumour with a usually poor prognosis, characterized by the proliferation of small cleaved lymphocytes with a diffuse growth pattern. We report a polymerase chain reaction-based analysis of minimal residual disease in a patient who achieved complete remission after allogeneic bloo...

journal_title：British journal of haematology

authors： Corradini P,Ladetto M,Astolfi M,Voena C,Tarella C,Bacigalupo A,Pileri A

更新日期：1996-08-01 00:00:00

abstract：:Givinostat, a histone-deacetylase inhibitor (HDACi), inhibits proliferation of cells bearing the JAK2 V617F mutation and has shown significant activity with good tolerability in patients with chronic myeloproliferative neoplasms (MPN). In this multicentre, open-label, phase II study, 44 patients with polycythaemia ver...

journal_title：British journal of haematology

authors： Finazzi G,Vannucchi AM,Martinelli V,Ruggeri M,Nobile F,Specchia G,Pogliani EM,Olimpieri OM,Fioritoni G,Musolino C,Cilloni D,Sivera P,Barosi G,Finazzi MC,Di Tollo S,Demuth T,Barbui T,Rambaldi A

更新日期：2013-06-01 00:00:00

abstract：:Thirty-two patients with meningeal leukaemia who achieved meningeal remission with intrathecal methotrexate (MTX) plus dexamethasone (DMT) were entered in a randomized study of two maintenance treatments: (a) I6 patients received intermittent intrathecal doses of MTX plus DMT, and (b) I6 patients received intermittent...

journal_title：British journal of haematology

authors： Muriel FS,Schere D,Barengols A,Eppinger-Helft M,Braier JL,Pavlovsky S,Macchi GH,Guman L

更新日期：1976-09-01 00:00:00

abstract：UNLABELLED:The action of clopidogrel on platelet receptors was analysed using platelets obtained from 11 healthy volunteers given 75 mg of clopidogrel daily for 8 d. Samples of blood were taken before treatment and after 8 d of medication. Determination of 2-methylthioadenosine diphosphate trisodium (2MesADP)-induced p...

journal_title：British journal of haematology

authors： Contreres JO,Dupuy E,Job B,Habib A,Bryckaert M,Rosa JP,Simoneau G,Herbert JM,Savi P,Levy-Toledano S

更新日期：2003-02-01 00:00:00

abstract：:Tissue factor pathway inhibitor (TFPI) is released to circulating blood after intravenous (i.v.) and subcutaneous (s.c.) injections of heparins, and may thus contribute to the antithrombotic effect of heparins. We have recently shown that total TFPI activity, plasma free TFPI antigen, and heparin releasable TFPI were ...

journal_title：British journal of haematology

authors： Bendz B,Hansen JB,Andersen TO,Ostergaard P,Sandset PM

更新日期：1999-12-01 00:00:00

abstract：:A number of studies have demonstrated induction of the unfolded protein response (UPR) in patients with severe congenital neutropenia (CN) harbouring mutations of ELANE, encoding neutrophil elastase. Why UPR is not activated in patients with cyclic neutropenia (CyN) carrying the same ELANE mutations is unclear. We eva...

journal_title：British journal of haematology

authors： Nustede R,Klimiankou M,Klimenkova O,Kuznetsova I,Zeidler C,Welte K,Skokowa J

更新日期：2016-01-01 00:00:00

abstract：:We assessed the role of human CD52 antibody (Campath-1H) in six patients with chronic lymphocytic leukaemia (CLL) treated to maximal response with purine analogues (fludarabine/deoxycoformycin) in whom persistent leukaemic infiltration of blood and bone marrow had precluded autologous stem cell transplantation. Five p...

journal_title：British journal of haematology

authors： Dyer MJ,Kelsey SM,Mackay HJ,Emmett E,Thornton P,Hale G,Waldmann H,Newland AC,Catovsky D

更新日期：1997-06-01 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：:Prospective studies in children with B-precursor acute lymphoblastic leukaemia (ALL) have shown that polymerase chain reaction (PCR)-based detection of minimal residual disease (MRD) using immunoglobin (Ig) and T-cell receptor (TCR) gene rearrangements as targets can be used to identify patients with a high relapse ri...

journal_title：British journal of haematology

authors： de Haas V,Breunis WB,Dee R,Verhagen OJ,Kroes W,van Wering ER,van Dongen JJ,van den Berg H,van der Schoot CE

更新日期：2002-01-01 00:00:00

abstract：:Bone disease in patients with thalassaemia major is a multifactorial and still poorly understood process. The present study evaluated 45 thalassaemic patients using dual X-ray absorptiometry at three sites (lumbar spine, head of femur and forearm) to assess bone mineral density, in parallel with a series of biochemica...

journal_title：British journal of haematology

authors： Voskaridou E,Kyrtsonis MC,Terpos E,Skordili M,Theodoropoulos I,Bergele A,Diamanti E,Kalovidouris A,Loutradi A,Loukopoulos D

更新日期：2001-01-01 00:00:00

abstract：:The expression and prognostic significance of hepatocyte growth factor (HGF) and its receptor c-MET (MET proto-oncogene) was analysed in 96 cases of diffuse large B-cell lymphoma (DLBCL). Tissue sections were immunohistochemically stained for HGF and c-Met. The prognosis of HGF-positive and c-Met-positive cases was si...

journal_title：British journal of haematology

authors： Kawano R,Ohshima K,Karube K,Yamaguchi T,Kohno S,Suzumiya J,Kikuchi M,Tamura K

更新日期：2004-11-01 00:00:00