Haemophilia B Liverpool: a new British family with mild haemophilia B associated with a -6 G to A mutation in the factor IX promoter.

abstract：:A cohort of 341 symptomless anti-human immunodeficiency virus (HIV)-1 positive individuals was screened over a 6-year period to detect a serum monoclonal gammopathy (MG) and to approach the prognostic significance of such anomaly in HIV infection. Eleven individuals with a MG were followed-up over a mean period of 50 ...

journal_title：British journal of haematology

authors： Lefrère JJ,Debbia M,Lambin P

更新日期：1993-05-01 00:00:00

abstract：:Besides circulating in normal plasma, von Willebrand factor (VWF) is also stored at relatively high concentration within the alpha-granules of platelets. This pool of platelet VWF exists distinct from plasma VWF, and is enriched in haemostatically-active high molecular weight multimers. Interestingly, the glycosylatio...

journal_title：British journal of haematology

authors： McGrath RT,McRae E,Smith OP,O'Donnell JS

更新日期：2010-03-01 00:00:00

abstract：:Budd-Chiari syndrome and portal vein thrombosis (BCS/PVT) are frequently associated with polycythaemia vera (PV). In an attempt to elucidate the mechanisms of BCS/PVT secondary to PV (T-PV), CD11b neutrophil expression, neutrophil oxidative burst and platelet-neutrophil complexes (PNC) were assessed in 17 such patient...

journal_title：British journal of haematology

authors： Alvarez-Larrán A,García-Pagán JC,Abraldes JG,Arellano E,Reverter JC,Bosch J,Cervantes F

更新日期：2004-02-01 00:00:00

abstract：:Peripheral blood haematopoietic progenitor cell mobilization has become a standard procedure prior to autologous stem cell transplantation. Biosimilar granulocyte colony-stimulating factors (GCSF) have recently been awarded European Union (EU) licences for stem cell mobilization but data for their use in this context ...

journal_title：British journal of haematology

authors： Publicover A,Richardson DS,Davies A,Hill KS,Hurlock C,Hutchins D,Jenner MW,Johnson PW,Lamb J,Launders H,McKeag N,Newman J,Orchard KH

更新日期：2013-07-01 00:00:00

abstract：:Podocalyxin-like protein (PCLP) is a sialomucin-type membrane protein structurally related to CD34 and endoglycan. It was first described in glomerular podocytes and endothelial cells. In mice, PCLP is present in haemangioblasts, and in both chicken and mice it is a marker of early haematopoietic stem cells and lineag...

journal_title：British journal of haematology

authors： Kerosuo L,Juvonen E,Alitalo R,Gylling M,Kerjaschki D,Miettinen A

更新日期：2004-03-01 00:00:00

abstract：:Treatment-free remission (TFR) has recently emerged as a goal of treatment in chronic myeloid leukaemia (CML). Molecular remission is sustained in around 30% of imatinib-treated patients who stop treatment after ≥2 years with undetectable minimal residual disease (UMRD) by conventional real-time reverse transcription ...

journal_title：British journal of haematology

authors： Ross DM,Hughes TP

更新日期：2014-07-01 00:00:00

abstract：:Myelofibrosis with myeloid metaplasia (MMM) is uniquely characterized by macroscopic bone marrow stromal changes that are believed to be both reactive and cytokine mediated. Furthermore, a prognostically detrimental increase in bone marrow angiogenesis has recently been demonstrated. These observations suggest a poten...

journal_title：British journal of haematology

authors： Elliott MA,Mesa RA,Li CY,Hook CC,Ansell SM,Levitt RM,Geyer SM,Tefferi A

更新日期：2002-05-01 00:00:00

abstract：:The impact of age, ethnicity and socio-economic deprivation in the era of novel anti-myeloma agents is unclear. Using linked national data from New Zealand, we evaluated the incidence, prevalence and overall survival (OS) of individuals who were diagnosed with myeloma between 2004 and 2016. The crude incidence rate in...

journal_title：British journal of haematology

authors： Chan HSH,Milne RJ

更新日期：2020-03-01 00:00:00

abstract：:Among 750 previously untreated patients with multiple myeloma, 27 (4%) presented with plasma cell leukaemia. All but one patient had high tumour mass and, when compared with comparable patients without leukaemia, more frequent extraosseous involvement, thrombocytopenia, high serum lactate dehydrogenase and hypodiploid...

journal_title：British journal of haematology

authors： Dimopoulos MA,Palumbo A,Delasalle KB,Alexanian R

更新日期：1994-12-01 00:00:00

abstract：:Platelet and cell stimulation lead to plasma membrane remodelling, resulting in phosphatidylserine (PS) externalisation in the outer leaflet of the membrane, associated with the shedding of PS-rich microparticles (MPs), and contributes to thrombin generation by promoting the assembly of coagulation enzyme complexes. A...

journal_title：British journal of haematology

authors： Proulle V,Hugel B,Guillet B,Grunebaum L,Lambert T,Freyssinet JM,Dreyfus M

更新日期：2005-11-01 00:00:00

abstract：:We have studied 61 patients who underwent allogeneic bone marrow transplantation (BMT) for chronic myeloid leukaemia (CML) in first chronic phase. Minimal residual disease was detected by the amplification of the leukaemia-specific BCR-ABL fusion mRNA with the polymerase chain reaction (PCR) using a highly sensitive n...

journal_title：British journal of haematology

authors： Cross NC,Hughes TP,Feng L,O'Shea P,Bungey J,Marks DI,Ferrant A,Martiat P,Goldman JM

更新日期：1993-05-01 00:00:00

abstract：:Peripheral blood globin synthesis studies were done in 11 patients with delta beta-thalassaemia trait, Hb S-delta beta-thalassaemia or delta beta/betao-thalassaemia from two black and two Caucasian families. All patients had elevated Hb F and normal or decreased Hb A2 levels and 10 had family studies confirming the di...

journal_title：British journal of haematology

authors： Kinney TR,Friedman S,Cifuentes E,Kim HC,Schwartz E

更新日期：1978-01-01 00:00:00

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：:This study assessed the incidence of immune thrombocytopenia (ITP) and characteristics associated with ITP in the paediatric population using the General Practice Research Database (GPRD). Two hundred and fifty-seven paediatric ITP patients were identified out of 1145 incident patients with ITP recorded between 1990 a...

journal_title：British journal of haematology

authors： Yong M,Schoonen WM,Li L,Kanas G,Coalson J,Mowat F,Fryzek J,Kaye JA

更新日期：2010-06-01 00:00:00

abstract：:The combined incidence of classical Philadelphia-negative myeloproliferative neoplasm (MPN) is 6-9/100 000 with a peak frequency between 50 and 70 years. MPN is less frequent in women of reproductive age. However, for essential thrombocythaemia (ET) in particular there is a second peak in women of reproductive age and...

journal_title：British journal of haematology

authors： Robinson SE,Harrison CN

更新日期：2020-05-01 00:00:00

abstract：:We examined major histocompatibility complex (MHC) class II expression in B cells, peripheral blood monocytes, activated T cells, epidermal Langerhans cells, monocyte-derived dendritic cells, dermal microvascular endothelial cells (DMEC) and fibroblasts of twin brothers with MHC class II deficiency. Although residual ...

journal_title：British journal of haematology

authors： Wolf HM,Thon V,Gulle H,Lechleitner S,Eibl MM,Petzelbauer P

更新日期：2001-11-01 00:00:00

abstract：:Mantle cell lymphoma (MCL) is an incurable mature B cell proliferation, combining the unfavourable clinical features of aggressive and indolent lymphomas. The blastic variant of MCL has an even worse prognosis and new treatment options are clearly needed. We analysed the effects of BL22, an immunotoxin composed of the...

journal_title：British journal of haematology

authors： Bogner C,Dechow T,Ringshausen I,Wagner M,Oelsner M,Lutzny G,Licht T,Peschel C,Pastan I,Kreitman RJ,Decker T

更新日期：2010-01-01 00:00:00

abstract：:Platelet antibodies either bound to the surface of platelets or free in the serum were sought in patients who had low platelet counts for a variety of reasons. They were detected by finding excess IgG on the surface of washed platelets either directly or after incubation of the serum with normal platelets. The techniq...

journal_title：British journal of haematology

authors： Hegde UM,Gordon-Smith EC,Worlledge S

更新日期：1977-01-01 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：:Interleukin-10 (IL-10) is one of the cytokines implicated in the pathogenesis of diffuse large B-cell lymphoma (DLBCL) in which it acts as auto/paracrine growth factor for lymphoma growth. T-cell non-Hodgkin lymphoma (NHL) is a heterogeneous disease, the biological basis of which is not fully understood. Some evidence...

journal_title：British journal of haematology

authors： Lee JJ,Kim DH,Lee NY,Sohn SK,Kim JG,Kim HJ,Do YR,Park YH

更新日期：2007-05-01 00:00:00

abstract：:A 14-month-old child who had a haemolytic episode when he was 5 years old, and with psychomotor retardation, was found to have decreased red cell hexokinase activity. The mutant enzyme was characterized by an increased affinity for glucose associated with an increased inhibition constant for glucose-1,6-diphosphate. A...

journal_title：British journal of haematology

authors： Magnani M,Stocchi V,Canestrari F,Dachà M,Balestri P,Farnetani MA,Giorgi D,Fois A,Fornaini G

更新日期：1985-09-01 00:00:00

abstract：:Lack of consensus for first-line marginal zone lymphoma (MZL) treatment and toxicities associated with currently available systemic therapies have inspired evaluation of immunotherapeutic agents yielding robust outcomes with improved tolerability. We previously reported durable efficacy with first-line lenalidomide an...

journal_title：British journal of haematology

authors： Becnel MR,Nastoupil LJ,Samaniego F,Davis RE,You MJ,Green M,Hagemeister FB,Fanale MA,Fayad LE,Westin JR,Wang M,Oki Y,Forbes SG,Feng L,Neelapu SS,Fowler NH

更新日期：2019-06-01 00:00:00

abstract：:The maintenance of physiological levels of haemoglobin is beneficial in the management of patients with thalassaemia major since it leads to better tissue oxygenation, reduction of blood volume, and reduced intestinal absorption of iron. We have studied 11 patients with thalassaemia major while treating them for 4-12 ...

journal_title：British journal of haematology

authors： Masera G,Terzoli S,Avanzini A,Fontanelli G,Mauri RA,Piacentini G,Ferrari M

更新日期：1982-09-01 00:00:00

abstract：:Leucine-rich repeats are conserved structural motifs present in the four components of the human platelet glycoprotein Ib/IX/V complex receptor for the adhesive protein von Willebrand factor. The absence or abnormality of this complex is responsible for Bernard-Soulier disease, an autosomal recessive bleeding disorder...

journal_title：British journal of haematology

authors： de la Salle C,Baas MJ,Lanza F,Schwartz A,Hanau D,Chevalier J,Gachet C,Briquel ME,Cazenave JP

更新日期：1995-02-01 00:00:00

abstract：:Although individuals with sickle cell anaemia (SCA) have elevated baseline inflammation and endothelial activation, the acute phase response to maximal exercise has not been evaluated among children with SCA. We measured the acute phase response to maximal exercise testing for soluble vascular cell adhesion molecule (...

journal_title：British journal of haematology

authors： Liem RI,Onyejekwe K,Olszewski M,Nchekwube C,Zaldivar FP,Radom-Aizik S,Rodeghier MJ,Thompson AA

更新日期：2015-12-01 00:00:00

abstract：:Screening of the skeleton by plain radiography was compared to magnetic resonance imaging (MRI) of the lumbar spine in 41 patients with multiple myeloma. In the lumbar spine, myeloma lesions were detected in 15 patients with radiography and in 28 patients with MRI. Radiography of the lumbar spine was not positive in a...

journal_title：British journal of haematology

authors： Tertti R,Alanen A,Remes K

更新日期：1995-11-01 00:00:00

abstract：:Early detection of platelet activation is important for the diagnosis and follow-up of several pathological conditions that primarily or secondarily involve platelets in their pathogenesis. The golden standard assay to detect thrombocyte activation is represented by the release of serotonin, classically performed by d...

journal_title：British journal of haematology

authors： Gobbi G,Mirandola P,Tazzari PL,Ricci F,Caimi L,Cacchioli A,Papa S,Conte R,Vitale M

更新日期：2003-06-01 00:00:00

abstract：:Cord blood (CB) has successfully been used as a stem cell source for haemopoietic reconstitution. However, a significant delay in platelet engraftment is consistently found in CB versus adult peripheral blood (PB) or bone marrow transplants. We sought to determine whether or not CB megakaryocytes have reached terminal...

journal_title：British journal of haematology

authors： Bornstein R,García-Vela J,Gilsanz F,Auray C,Calés C

更新日期：2001-08-01 00:00:00

abstract：:The Complement 1 trial investigated the efficacy and safety of ofatumumab + chlorambucil with chlorambucil monotherapy in patients with previously untreated chronic lymphocytic leukaemia (CLL). On long-term follow-up in the chemoimmunotherapy arm vs. the chemotherapy arm there was an estimated 12% (not significant) an...

journal_title：British journal of haematology

authors： Offner F,Robak T,Janssens A,Govind Babu K,Kloczko J,Grosicki S,Mayer J,Panagiotidis P,Schuh A,Pettitt A,Montillo M,Werner O,Vincent G,Khanna S,Hillmen P

更新日期：2020-09-01 00:00:00

abstract：:There is little information published comparing peripheral blood stem cells (PBSC) with bone marrow (BM) as the stem cell source in the long-term outcome in recipients of T-cell depleted (TCD) unrelated donor (UD) transplants. We present retrospective outcome data on 306 recipients of myeloablative, human leucocyte an...

journal_title：British journal of haematology

authors： Shaw BE,Apperley JF,Russell NH,Craddock C,Liakopoulou E,Potter MN,Wynn R,Gibson B,Pearce RM,Kirkland K,Lee J,Madrigal JA,Cook G,Byrne JL

更新日期：2011-04-01 00:00:00