Abnormal platelet function in Chediak-Higashi syndrome.

abstract：:Twenty-six members of four generations of one family in which a man was diagnosed in 1961 as having von Willebrand's disease (vWD) have been studied. Subtype IIB vWD and autosomal dominant inheritance was identified in 19 individuals with bleeding signs varying in severity and frequency. The absence of high molecular ...

journal_title：British journal of haematology

authors： Mazurier C,Parquet-Gernez A,Goudemand J,Taillefer MF,Goudemand M

更新日期：1988-08-01 00:00:00

abstract：:New therapeutic approaches are needed to improve the cure rates in acute myeloid leukaemia (AML). The present study was designed to investigate whether: (1) cytotoxic lymphocytes could be expanded from AML patients in complete remission; (2) their signal transduction machinery was preserved; (3) these cells were capab...

journal_title：British journal of haematology

authors： Torelli GF,Guarini A,Palmieri G,Breccia M,Vitale A,Santoni A,Foa R

更新日期：2002-02-01 00:00:00

abstract：:Coagulation activity and whole-blood viscosity were measured in the steady state, and serially during painful crisis, in eight patients with sickle-cell anaemia. Platelet and coagulation activation occurred in the steady state and became more pronounced early in crisis. Whole-blood viscosity increased during crisis in...

journal_title：British journal of haematology

authors： Richardson SG,Matthews KB,Stuart J,Geddes AM,Wilcox RM

更新日期：1979-01-01 00:00:00

abstract：:Six hundred and nineteen patients with de novo acute myeloid leukaemia, entered into the Medical Research Council's eighth trial of therapy have been studied. All patients were treated with the same remission induction regimen. Pretreatment variables comprising age, clinical status, haematological status and a detaile...

journal_title：British journal of haematology

authors： Swirsky DM,de Bastos M,Parish SE,Rees JK,Hayhoe FG

更新日期：1986-11-01 00:00:00

abstract：:The discovery of two different types of alpha globin gene quadruplication is reported. One with the alpha alpha alpha alpha (anti 3.7)/haplotype was present in four members of a Black family from Georgia, while a second with the alpha alpha alpha alpha (anti 4.2)/haplotype was observed in two members of an Indonesian ...

journal_title：British journal of haematology

authors： Gu YC,Landman H,Huisman TH

更新日期：1987-06-01 00:00:00

abstract：:Pericardial effusion (PE) is a rare but potentially life-threatening complication for allogeneic haematopoietic stem cell transplantation (HSCT) recipients. The risk factors, aetiology, incidence and therapy are largely unclear. To investigate this issue, we reviewed 391 adult patients undergoing allogeneic HSCT betwe...

journal_title：British journal of haematology

authors： Liu YC,Chien SH,Fan NW,Hu MH,Gau JP,Liu CJ,Yu YB,Liu CY,Hsiao LT,Liu JH,Chiou TJ,Tzeng CH

更新日期：2015-06-01 00:00:00

abstract：:Erythroid regeneration is an important and separate element in the engraftment process in allogeneic and autologous bone marrow transplantation (alloBMT, autoBMT). Qualitative visual reticulocyte counting has proved inadequate in the evaluation of erythropoiesis after BMT but automated flow cytometry now allows the re...

journal_title：British journal of haematology

authors： Davies SV,Cavill I,Bentley N,Fegan CD,Poynton CH,Whittaker JA

更新日期：1992-05-01 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：:The development of neutralising antibodies to factor VIII (FVIII) is a major complication of haemophilia A (HA) therapy. We aimed to construct an individual risk profile for the development of inhibitors in HA and started by screening for the causative mutation in our HA patient population. A total of 109 patients and...

journal_title：British journal of haematology

authors： Boekhorst J,Verbruggen B,Lavergne JM,Costa JM,Schoormans SC,Brons PP,van Kraaij MG,Nováková IR,van Heerde WL

更新日期：2005-10-01 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：:It has been suggested that in blast crisis (BC) of chronic myeloid leukaemia (CML) the clinical and laboratory features of patients with 'lymphoid' phenotype differ from those of patients with non-lymphoid BC. In order to assess any differences, 97 patients consecutively diagnosed with BC that followed a known chronic...

journal_title：British journal of haematology

authors： Cervantes F,Villamor N,Esteve J,Montoto S,Rives S,Rozman C,Montserrat E

更新日期：1998-01-01 00:00:00

abstract：:Laboratory screening for lupus anticoagulant (LA) has been shown to be suboptimal in several studies. Guidelines have recently been published by an expert group for the British Committee for Standards in Haematology, in an attempt to standardize and improve screening procedures. The value of using screening tests conf...

journal_title：British journal of haematology

authors： Jennings I,Greaves M,Mackie IJ,Kitchen S,Woods TA,Preston FE,UK National External Quality Assessment Scheme for Blood Coagulation.

更新日期：2002-11-01 00:00:00

abstract：:Recently, leukaemia-associated antigens (LAA) recognized by T lymphocytes, such as Wilm's tumour-1 (WT-1) or pathogenesis-related protein-1 (PR-1), have been identified. For immunotherapies that employ antigen peptides, either alone or pulsed on dendritic cells (DC), the expression of human leucocyte antigen (HLA) mol...

journal_title：British journal of haematology

authors： Vollmer M,Li L,Schmitt A,Greiner J,Reinhardt P,Ringhoffer M,Wiesneth M,Döhner H,Schmitt M

更新日期：2003-03-01 00:00:00

abstract：:Restriction endonuclease analysis has been performed on the alpha and beta globin gene clusters of 57 Cypriots homozygous for beta thalassaemia, 30 with the transfusion dependent form of the condition (thalassaemia major) and 27 who are less severely affected (thalassaemia intermedia). There was a significant differen...

journal_title：British journal of haematology

authors： Wainscoat JS,Kanavakis E,Wood WG,Letsky EA,Huehns ER,Marsh GW,Higgs DR,Clegg JB,Weatherall DJ

更新日期：1983-03-01 00:00:00

abstract：:Mutations in SOCS1 are frequent in primary mediastinal B-cell lymphoma and classical Hodgkin lymphoma. In the latter, SOCS1 mutations affect the length of the encoded protein (major mutations) and are associated with shorter patient survival. Two independent studies examined the prognostic impact of SOCS1 mutations in...

journal_title：British journal of haematology

authors： Mellert K,Martin M,Lennerz JK,Lüdeke M,Staiger AM,Kreuz M,Löffler M,Schmitz N,Trümper L,Feller AC,Hartmann S,Hansmann ML,Klapper W,Stein H,Rosenwald A,Ott G,Ziepert M,Möller P

更新日期：2019-12-01 00:00:00

abstract：:During definitive erythropoiesis, erythroid precursors undergo differentiation through multiple nucleated states to an enucleated reticulocyte, which loses its residual RNA/organelles to become a mature erythrocyte. Over the course of these transformations, continuous changes in membrane proteins occur, including shif...

journal_title：British journal of haematology

authors： Kodippili GC,Spector J,Kang GE,Liu H,Wickrema A,Ritchie K,Low PS

更新日期：2010-09-01 00:00:00

abstract：:Immune-mediated pathways have been recognized to be of importance in the pathogenesis of chronic lymphocytic leukaemia (CLL). We assessed a broad variety of immune-related and inflammatory conditions and subsequent CLL development among 4 million adult male veterans admitted to VA hospitals. We identified 3,680 CLL ca...

journal_title：British journal of haematology

authors： Landgren O,Gridley G,Check D,Caporaso NE,Morris Brown L

更新日期：2007-12-01 00:00:00

abstract：:Whether or not pregnant women with a previous episode of venous thromboembolism (VTE) should receive antithrombotic prophylaxis is a matter of debate. In order to estimate the rate of recurrent deep venous thrombosis (DVT) or pulmonary embolism (PE) during pregnancy and puerperium we retrospectively investigated a coh...

journal_title：British journal of haematology

authors： De Stefano V,Martinelli I,Rossi E,Battaglioli T,Za T,Mannuccio Mannucci P,Leone G

更新日期：2006-11-01 00:00:00

abstract：:A case of acute myeloid leukaemia (AML) occurring in a patient with untreated chronic lymphatic leukaemia (CLL) is presented. The diagnosis of two simultaneous leukaemic processes is based on morphological, cytochemical and immunological findings. The significance of the development of AML in patients with CLL is disc...

journal_title：British journal of haematology

authors： Lawlor E,McCann SR,Whelan A,Greally J,Temperley IJ

更新日期：1979-11-01 00:00:00

abstract：:To investigate the role of retinoblastoma susceptibility (RB) gene inactivation in leukaemogenesis, we evaluated 36 bone marrow specimens of acute leukaemia for RB protein expression by immunoprecipitation and Western blot analysis. 15 patients had no detectable RB protein at initial screening. However, nine of them w...

journal_title：British journal of haematology

authors： Tang JL,Yeh SH,Chen PJ,Lin MT,Tien HF,Chen YC

更新日期：1992-11-01 00:00:00

abstract：:Tumour necrosis factor-α (TNF) is an inflammatory cytokine that is elevated in a number of autoimmune diseases including immune thrombocytopenia (ITP), a bleeding disorder characterized by low platelet counts. In vitro TNF blockade increases expansion of the regulatory T cell (Treg) IKZF2 (also termed Helios) subset i...

journal_title：British journal of haematology

authors： Zhong H,Bussel J,Yazdanbakhsh K

更新日期：2015-01-01 00:00:00

abstract：:Polycythaemia vera (PV) patients have an overall comparatively favourable prognosis, but disease progression is very heterogeneous and life-threatening thrombosis and bleedings are frequent complications in untreated disease. Moreover, transformation to more severe secondary myelofibrosis and acute myeloid leukaemia c...

journal_title：British journal of haematology

authors： Andréasson B,Pettersson H,Wasslavik C,Johansson P,Palmqvist L,Asp J

更新日期：2020-06-01 00:00:00

abstract：:Platelet function was evaluated in eight patients with chronic granulocytic leukaemia (CGL), seven Ph1 positive and one Ph1 negative. Seven of the eight patients' platelets had an absence of the second wave of adrenaline induced aggregation on at least one occasion, while five had impaired collagen aggregation. The pl...

journal_title：British journal of haematology

authors： Gerrard JM,Stoddard SF,Shapiro RS,Coccia PF,Ramsay NK,Nesbit ME,Rao GH,Krivit W,White JG

更新日期：1978-12-01 00:00:00

abstract：:Recurrent large-scale somatic copy number alterations (SCNAs), and somatic point mutations can be analysed to stratify patients with chronic lymphocytic leukaemia (CLL) into distinct prognostic groups. To investigate the relationship between SCNAs and somatic mutations, we performed whole-exome sequencing and single n...

journal_title：British journal of haematology

authors： Zhou W,Goldin L,Wang M,McMaster ML,Jones K,Burdett L,Chanock SJ,Yeager M,Dean M,Caporaso NE

更新日期：2018-06-01 00:00:00

abstract：:Since the description in 1974 of the 5q--syndrome, only 29 cases have been reported. Over a 3 1/2 year period cytogenetic culture of bone marrow submitted from 344 patients being investigated for a blood disorder revealed nine patients with anomalies of chromosome 5. In five of these patients (samples arising from 37 ...

journal_title：British journal of haematology

authors： Tinegate H,Gaunt L,Hamilton PJ

更新日期：1983-05-01 00:00:00

abstract：:We describe three patients with chronic granulocytic leukaemia who developed impaired visual acuity in association with high leucocyte counts. Two of the patients were in the chronic phase of their disease and the third developed visual symptoms at the time of blastic transformation. Fundoscopy showed retinal haemorrh...

journal_title：British journal of haematology

authors： Mehta AB,Goldman JM,Kohner E

更新日期：1984-04-01 00:00:00

abstract：:We report a Lancashire family with mild haemophilia B associated with a -6 G to A mutation in the factor IX promoter. This mutation has been reported previously in Britain in one other affected family. The factor IX haplotype of these two unrelated patients was determined by PCR analysis of seven polymorphic sites wit...

journal_title：British journal of haematology

authors： Stowell KM,Figueiredo MS,Brownlee GG,Jones P,Bolton-Maggs PH

更新日期：1993-09-01 00:00:00

abstract：:The PFA-100(R) (PFA) diagnostic system for the detection of platelet dysfunction was evaluated to determine reference ranges in a normal population. The PFA determines the primary haemostasis capacity (PHC) of anticoagulated whole blood, expressed by the system's closure time (CT). In this study the CT reference range...

journal_title：British journal of haematology

authors： Böck M,De Haan J,Beck KH,Gutensohn K,Hertfelder HJ,Karger R,Heim MU,Beeser H,Weber D,Kretschmer V

更新日期：1999-09-01 00:00:00

abstract：:Four patients with paroxysmal nocturnal haemoglobinuria (PNH) were treated with cyclosporine. The treatment with cyclosporine was based on the hypothesis that immune-mediated bone-marrow damage is the common pathogenetic mechanism of aplasia and PNH, with lack of GPI-linked ligands for an immune attack (i.e. LFA-3, CD...

journal_title：British journal of haematology

authors： van Kamp H,van Imhoff GW,de Wolf JT,Smit JW,Halie MR,Vellenga E

更新日期：1995-01-01 00:00:00

abstract：:We performed a retrospective study analysing the effect of sorafenib, an oral fms-Like Tyrosine Kinase 3 (FLT3)/multikinase inhibitor, as post-transplant maintenance in adult patients with FLT3-internal tandem duplication (ITD) acute myeloid leukaemia (AML). We identified consecutive patients with FLT3-ITD AML diagnos...

journal_title：British journal of haematology

authors： Brunner AM,Li S,Fathi AT,Wadleigh M,Ho VT,Collier K,Connolly C,Ballen KK,Cutler CS,Dey BR,El-Jawahri A,Nikiforow S,McAfee SL,Koreth J,Deangelo DJ,Alyea EP,Antin JH,Spitzer TR,Stone RM,Soiffer RJ,Chen YB

更新日期：2016-11-01 00:00:00