Red cell pyruvate kinase deficiency: molecular and clinical aspects.

abstract：:We report a case of persistent infection with human parvovirus B19 (PVB19), manifesting clinically as recurrent agranulocytosis and in bone marrow biopsy as recurrent pure granulocytic aplasia. Persistence of parvovirus infection was documented by the presence of PVB19 DNA and anti-PVB19-IgM antibodies in the serum fo...

journal_title：British journal of haematology

authors： Pont J,Puchhammer-Stöckl E,Chott A,Popow-Kraupp T,Kienzer H,Postner G,Honetz N

更新日期：1992-02-01 00:00:00

abstract：:The haematological changes in early years following neonatal diagnosis have been observed in representative groups of children with sickle cell-haemoglobin C (SC) disease, sickle cell-beta(+) thalassaemia, and in sickle cell-beta(0) thalassaemia. Most haematological indices in SC disease were intermediate between prev...

journal_title：British journal of haematology

authors： Stevens MC,Maude GH,Beckford M,Grandison Y,Mason K,Serjeant BE,Taylor B,Topley JM,Serjeant GR

更新日期：1985-06-01 00:00:00

abstract：:We have previously shown that allogeneic bone marrow transplantation (BMT) with cryopreserved donor marrow cells can be used without prolonging the engraftment time or interfering with the reconstitution of haemopoiesis. In this report we extend our initial observations of the first 40 patients who underwent allogenei...

journal_title：British journal of haematology

authors： Stockschläder M,Hassan HT,Krog C,Krüger W,Löliger C,Horstman M,Altnöder M,Clausen J,Grimm J,Kabisch H,Zander A

更新日期：1997-02-01 00:00:00

abstract：:Monoclonal B-cell populations have been detected in the peripheral blood of apparently healthy individuals by flow cytometry. In 2005, the term monoclonal B-cell lymphocytosis (MBL) was proposed to describe these findings. MBL may be immunophenotypically similar to chronic lymphocytic leukaemia (CLL) and, like CLL, th...

journal_title：British journal of haematology

authors： Rachel JM,Zucker ML,Fox CM,Plapp FV,Menitove JE,Abbasi F,Marti GE

更新日期：2007-12-01 00:00:00

abstract：:Hepatitis E virus (HEV) is increasingly found to cause hepatitis in allogeneic haematopoietic stem cell transplantation (HSCT) patients. However, little is known about HEV infection in patients receiving haploidentical HSCT (haplo-HSCT). Here, we retrospectively evaluate the incidence and clinical course of HEV infect...

journal_title：British journal of haematology

authors： Tang FF,Mo XD,Wang Y,Yan CH,Chen YH,Chen H,Han W,Chang YJ,Zhang HY,Xie YD,Ma H,Wei L,Xu LP,Huang XJ,Zhang XH

更新日期：2019-03-01 00:00:00

abstract：:The use of peripheral blood stem cells instead of bone marrow as the source of haemopoietic cells for allogeneic transplantation is being increasingly explored. We have analysed data from 17 normal donors who underwent stem cell mobilization for allogeneic transplantation with an identical protocol using G-CSF at a do...

journal_title：British journal of haematology

authors： Miflin G,Charley C,Stainer C,Anderson S,Hunter A,Russell N

更新日期：1996-11-01 00:00:00

abstract：:An immunotoxin containing an anti-CD30 monoclonal antibody (Ber-H2) and saporin, a ribosome-inactivating protein type 1, is described. It specifically inhibits protein synthesis by Hodgkin derived target cell lines with a very high efficiency (IC50 ranging from 5 x 10(-12) M to 5 x 10(-14) M, as saporin), while irrele...

journal_title：British journal of haematology

authors： Tazzari PL,Bolognesi A,de Totero D,Falini B,Lemoli RM,Soria MR,Pileri S,Gobbi M,Stein H,Flenghi L

更新日期：1992-06-01 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：:In this study we have investigated the molecular basis for a mild form of beta-thalassaemia in three patients of Italian descent. In two, belonging to different families and affected by a mild and late-presenting form of thalassaemia major, direct sequencing of amplified DNA detected a C----T substitution at position ...

journal_title：British journal of haematology

authors： Meloni A,Rosatelli MC,Faà V,Sardu R,Saba L,Murru S,Sciarratta GV,Baldi M,Tannoia N,Vitucci A

更新日期：1992-02-01 00:00:00

abstract：:Diamond Blackfan Anaemia (DBA) is a rare congenital pure red cell aplasia that may be associated with facio-skeletal developmental defects. The disease is caused by mutations in one of at least ten ribosomal proteins, which results in haploinsufficiency and an imbalance between the synthesis of rRNA and ribosomal prot...

journal_title：British journal of haematology

authors： Horos R,von Lindern M

更新日期：2012-12-01 00:00:00

abstract：:Data on endogenous thrombopoietin (TPO) levels and their regulation in myelodysplastic syndromes (MDS) are sparse. We examined the plasma TPO level of 85 MDS patients by a sensitive enzyme immunoassay and the platelet expression of TPO receptor (TPO-R) protein, which metabolizes endogenous TPO, in 19 MDS patients with...

journal_title：British journal of haematology

authors： Tamura H,Ogata K,Luo S,Nakamura K,Yokose N,Dan K,Tohyama K,Yoshida Y,Hamaguchi H,Sakamaki H,Kuwaki T,Tahara T,Kato T,Nomura T

更新日期：1998-12-01 00:00:00

abstract：:SAn ageing population and the continuing expansion of clinical indications for coumadin therapy have increased pressure on hospital anticoagulant clinics. One solution is patient self-testing (PST) of the international normalized ratio (INR) using capillary blood samples on point-of-care coagulation monitors at home. ...

journal_title：British journal of haematology

authors： Gardiner C,Williams K,Mackie IJ,Machin SJ,Cohen H

更新日期：2005-01-01 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：: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：:The platelets of a young man with the grey platelet syndrome were severely depleted of all seven alpha-granule proteins assayed as well as partially deficient in alpha-mannosidase and alpha-fucosidase; four other lysosomal enzymes were present in normal concentrations. Total platelet 5-hydroxytryptamine (5HT) and aden...

journal_title：British journal of haematology

authors： Srivastava PC,Powling MJ,Nokes TJ,Patrick AD,Dawes J,Hardisty RM

更新日期：1987-04-01 00:00:00

abstract：:A 2-year-old girl is described with severe haemophilia A (factor VIII: C < 0.01 units/ml). Both of her parents were phenotypically normal. Cytogenetic analysis on the proband demonstrated an interstitial X chromosome deletion encompassing Xq26-q28. Molecular studies with several polymorphic markers close to and within...

journal_title：British journal of haematology

authors： Windsor S,Lyng A,Taylor SA,Ewenstein BM,Neufeld EJ,Lillicrap D

更新日期：1995-08-01 00:00:00

abstract：:An open, randomised study was performed to determine whether two doses of influenza vaccine were more effective than one to elicit an immune response in 70 patients with haematological malignancies. The responses were not improved by two doses compared with one (influenza A virus serotypes H1/N1 18% vs. 22% and H3/N2 ...

journal_title：British journal of haematology

authors： Ljungman P,Nahi H,Linde A

更新日期：2005-07-01 00:00:00

abstract：:Follow-up studies of natural killer (NK) cells, NK activity and antibody dependent cellular cytotoxicity (ADCC) in the course of hairy cell leukaemia (HCL) were carried out in a series of patients affected by the disease. NK activity against K562 targets was found to be high in all the patients with non-symptomatic st...

journal_title：British journal of haematology

authors： Demeter J,Pálóczi K,Lehoczky D,Benczúr M

更新日期：1989-02-01 00:00:00

abstract：:Two unrelated cases of congenital dyserythropoietic anaemia (CDA) are described. They show striking similarities which could not be attributed to one of the well-known types of CDA or any other congenital disease of the erythroid system. Both patients were followed for many years before and after splenectomy. There wa...

journal_title：British journal of haematology

authors： Bethlenfalvay NC,Hadnagy C,Heimpel H

更新日期：1985-07-01 00:00:00

abstract：:Minimal (i.e. submicroscopic) residual disease (MRD) predicts outcome in childhood acute lymphoblastic leukaemia (ALL). To be used clinically, MRD assays must be reliable and accurate. Two well-established techniques, flow cytometry (FC) and polymerase chain reaction (PCR), can detect leukaemic cells with a sensitivit...

journal_title：British journal of haematology

authors： Kerst G,Kreyenberg H,Roth C,Well C,Dietz K,Coustan-Smith E,Campana D,Koscielniak E,Niemeyer C,Schlegel PG,Müller I,Niethammer D,Bader P

更新日期：2005-03-01 00:00:00

abstract：:Pathogen reduction (PR) of platelet products increases costs and available clinical studies are equivocal with respect to clinical and haemostatic effectiveness. We conducted a multicentre, open-label, randomized, non-inferiority trial comparing the clinical effectiveness of buffy-coat derived leukoreduced platelet co...

journal_title：British journal of haematology

authors： Kerkhoffs JL,van Putten WL,Novotny VM,Te Boekhorst PA,Schipperus MR,Zwaginga JJ,van Pampus LC,de Greef GE,Luten M,Huijgens PC,Brand A,van Rhenen DJ,Dutch - Belgian HOVON cooperative group.

更新日期：2010-07-01 00:00:00

abstract：:The novel multi-kinase inhibitor TG02 has selectivity against cell cycle and transcriptional cyclin dependent kinases (CDKs) as well as fms-like tyrosine kinase receptor-3 (FLT3). Inhibition of transcriptional CDKs preferentially depletes short-lived proteins such as MCL1. We evaluated the in vitro toxicity of TG02 to...

journal_title：British journal of haematology

authors： Pallis M,Abdul-Aziz A,Burrows F,Seedhouse C,Grundy M,Russell N

更新日期：2012-10-01 00:00:00

abstract：:A chromogenic factor Xa generation method has been developed for comparing the co-factor activity of factor VIII concentrates at physiological factor VIII concentrations (1 iu/ml). In the presence of thrombin all concentrates gave similar rapid rates of factor Xa generation, but in the absence of thrombin there were m...

journal_title：British journal of haematology

authors： Kemball-Cook G,Tubbs JE,Dawson NJ,Barrowcliffe TW

更新日期：1993-06-01 00:00:00

abstract：:To explore the potential efficacy of l-asparaginase treatment in acute myeloid leukaemia (AML) patients, we studied the in vitro resistance of French-American-British (FAB) subtypes of childhood AML to l-asparaginase using a methyl-thiazol-tetrazolium assay. We tested leukaemic cells obtained from 177 common acute lym...

journal_title：British journal of haematology

authors： Okada S,Hongo T,Yamada S,Watanabe C,Fujii Y,Ohzeki T,Horikoshi Y,Ito T,Yazaki M,Komada Y,Tawa A

更新日期：2003-12-01 00:00:00

abstract：:The myelodysplastic syndromes (MDS) have a significant frequency of evolution into acute myeloid leukaemia (AML). Approximately 30% of MDS patients show activating mutations of the N-RAS proto-oncogene, and these patients are at increased risk of leukaemic evolution. Long-term survivors of aplastic anaemia (AA) and pa...

journal_title：British journal of haematology

authors： White JR,Josten KM,Chopra R,Tooze J,Saso R,Gordon-Smith EC,Rutherford TR

更新日期：1995-12-01 00:00:00

abstract：:Previously described platelet-aggregating antibodies associated with thrombosis and thrombocytopenia required heparin for their in vivo and in vitro expression. We have observed a patient with thrombosis who became thrombocytopenic during heparin treatment, but who suffered further thrombotic events and continued thro...

journal_title：British journal of haematology

authors： Pfueller SL,David R,Firkin BG,Bilston RA,Cortizo WF,Raines G

更新日期：1990-03-01 00:00:00

abstract：:Analogous to T cells, Natural Killer (NK) cells may facilitate engraftment, combat infection, and control cancer in bone marrow or haematopoietic stem cell transplantation (HSCT); however, NK cells do not cause graft-versus-host disease. Killer immunoglobulin-like receptors (KIRs) regulate NK cell function, and recent...

journal_title：British journal of haematology

authors： Leung W

更新日期：2011-10-01 00:00:00

abstract：:The thrombopoietin receptor agonists (THPO-RAs), romiplostim and eltrombopag, are effective and safe in immune thrombocytopenia (ITP). However, the value of their sequential use when no response is achieved or when adverse events occur with one THPO-RA has not been clearly established. Here we retrospectively evaluate...

journal_title：British journal of haematology

authors： González-Porras JR,Mingot-Castellano ME,Andrade MM,Alonso R,Caparrós I,Arratibel MC,Fernández-Fuertes F,Cortti MJ,Pascual C,Sánchez-González B,Bernat S,Fuertes-Palacio MA,Vázquez-Paganini JA,Olivera PE,Alvarez-Román MT,Jarq

更新日期：2015-04-01 00:00:00

abstract：:Bacteria in platelet components (PC) may result in transfusion-related sepsis (TRS). Pathogen inactivation of PC with amotosalen (A-PC) can abrogate the risk of TRS and hence facilitate storage to 7 d. A randomized, controlled, double-blinded trial to evaluate the efficacy and safety of A-PC stored for 6-7 d was condu...

journal_title：British journal of haematology

authors： Lozano M,Knutson F,Tardivel R,Cid J,Maymó RM,Löf H,Roddie H,Pelly J,Docherty A,Sherman C,Lin L,Propst M,Corash L,Prowse C

更新日期：2011-05-01 00:00:00

abstract：:Duffy blood group antigens are carried on a glycoprotein that is predicted to pass through the erythrocyte membrane seven times and is a promiscuous chemokine receptor. The Fy(a- b-) phenotype is present in two-thirds of African-American Blacks but is rare in Caucasians. In Blacks, the phenotype is due to a non-functi...

journal_title：British journal of haematology

authors： Rios M,Chaudhuri A,Mallinson G,Sausais L,Gomensoro-Garcia AE,Hannon J,Rosenberger S,Poole J,Burgess G,Pogo O,Reid M

更新日期：2000-02-01 00:00:00