Aberrant splicing of genes involved in haemoglobin synthesis and impaired terminal erythroid maturation in SF3B1 mutated refractory anaemia with ring sideroblasts.

abstract：:Human monocytes contain a series of alpha-naphthyl acetate (alpha NA) esterases which are not present in other blood cells and which can be specifically inhibited by bis(4-nitrophenyl)-phosphate (BNPP). This inhibitor is non-toxic at the concentration used and thus enabled studies on the possible significance of this ...

journal_title：British journal of haematology

authors： Oertel J,Hagner G,Kastner M,Huhn D

更新日期：1985-12-01 00:00:00

abstract：:Erythrocyte sedimentation, viscosity, electrophroetic mobility, and osmotic fragility were measured in a study of the rheological behaviour of the erythrocyte in vitro. In comparison with physiological saline solution, a suspending medium containing a gamma2lambda2 monoclonal IgG from a patient with multiple myeloma c...

journal_title：British journal of haematology

authors： Rovel A,Vigneron C,Streiff F

更新日期：1979-04-01 00:00:00

abstract：:Sixty patients with haematological malignancies received a myeloablative regimen of total body irradiation, cyclophosphamide and fludarabine followed by a T-cell-depleted peripheral blood stem cell transplant from a human leucocyte antigen identical sibling. To improve donor immune function, 1 x 10(7) CD3+ cells/kg we...

journal_title：British journal of haematology

authors： Montero A,Savani BN,Kurlander R,Read EJ,Leitman SF,Childs R,Solomon SR,Barrett AJ

更新日期：2005-09-01 00:00:00

abstract：:A multicentre modified World Health Organization (WHO)-type international sensitivity index (ISI) calibration has been performed at 10 European Concerted Action on Anticoagulation (ECAA) national laboratories using non-citrated whole-blood on two point-of-care test (POCT) prothrombin time (PT) monitor systems, CoaguCh...

journal_title：British journal of haematology

authors： Poller L,Keown M,Chauhan N,van Den Besselaar AM,Tripodi A,Jespersen J,Shiach C,Horellou MH,Dias D,Egberg N,Iriarte JA,Kontopoulou-Griva I,Otridge B

更新日期：2002-03-01 00:00:00

abstract：:We have investigated the potency of the C677T mutation in the methylene tetrahydrofolate reductase (MTHFR) gene as a genetic risk factor in women with a history of early (12 weeks gestation) recurrent miscarriage (three or more consecutive pregnancy losses). Fifty-seven of the tota...

journal_title：British journal of haematology

authors： Holmes ZR,Regan L,Chilcott I,Cohen H

更新日期：1999-04-01 00:00:00

abstract：:Enteric coated aspirin (ECA) at doses of 325-1300 mg is an effective alternative to regular aspirin for inhibition of platelet activity while avoiding gastric irritation. The objectives of this study were to determine: (1) the lowest chronic dose of ECA providing effective inhibition of platelet activities, (2) the ti...

journal_title：British journal of haematology

authors： Jakubowski JA,Stampfer MJ,Vaillancourt R,Deykin D

更新日期：1985-08-01 00:00:00

abstract：:Endothelial cells (EC) were isolated from the umbilical vein of a newborn girl with type I 'platelet low' von Willebrand disease (I vWD) and endothelial localization and release and the ability of subendothelial von Willebrand factor (vWF) to support platelet adhesion were compared with those of normal EC. vWF was det...

journal_title：British journal of haematology

authors： Federici AB,de Groot PG,Moia M,Ijsseldijk MJ,Sixma JJ,Mannucci PM

更新日期：1993-01-01 00:00:00

abstract：:MYD88 mutations are present in 95% of Waldenstrom Macroglobulinaemia (WM) patients, and support diagnostic discrimination from other IgM-secreting B-cell malignancies. Diagnostic discrimination can be difficult among suspected wild-type MYD88 (MYD88WT ) WM cases. We systematically reviewed the clinical, pathological a...

journal_title：British journal of haematology

authors： Treon SP,Gustine J,Xu L,Manning RJ,Tsakmaklis N,Demos M,Meid K,Guerrera ML,Munshi M,Chan G,Chen J,Kofides A,Patterson CJ,Yang G,Liu X,Severns P,Dubeau T,Hunter ZR,Castillo JJ

更新日期：2018-02-01 00:00:00

abstract：:To explore the possible role of a residual or variant alphaIIbbeta3 integrin (alphaIIbbeta3) in thrombogenesis, we used a new ex vivo perfusion chamber model to examine blood from patients with different subtypes of Glanzmann's thrombasthenia (GT). Non-anticoagulated blood was perfused through capillaries coated with ...

journal_title：British journal of haematology

authors： Hainaud P,Brouland JP,André P,Simoneau G,Bal Dit Sollier C,Drouet L,Caen J,Bellucci S

更新日期：2002-12-01 00:00:00

abstract：:The organelle pathology of neutrophils in chronic granulocytic leukaemia (CGL) was investigated by analytical subcellular fractionation. There were minor reductions in activity of some granule enzymes with an abnormal distribution in sucrose density gradients of the specific granules. There was a marked reduction of 5...

journal_title：British journal of haematology

authors： Rustin GJ,Peters TJ

更新日期：1979-04-01 00:00:00

abstract：:A number of disorders cause iron overload: some are of genetic origin, such as hereditary haemochromatosis, while others are acquired, for instance due to repeated transfusions. This article reviews the treatment options for hereditary haemochromatosis, with special attention to the use of erythrocytapheresis. In gene...

journal_title：British journal of haematology

authors： Rombout-Sestrienkova E,van Kraaij MG,Koek GH

更新日期：2016-12-01 00:00:00

abstract：:We describe two British families with similar, dominantly-inherited, temperature-related variants of hereditary stomatocytosis, consistent with the original description of 'cryohydrocytosis'. The cells show a 5-6-fold increase in passive permeability at 37 degrees C with abnormal intracellular Na and K levels at 15-20...

journal_title：British journal of haematology

authors： Coles SE,Chetty MC,Ho MM,Nicolaou A,Kearney JW,Wright SD,Stewart GW

更新日期：1999-06-01 00:00:00

abstract：:Unfractionated heparin (UFH) and low-molecular weight heparin (LMWH) are well defined anticoagulant agents. Recent data suggest that both LMWH and UFH may also have potent anti-inflammatory properties; however, their mechanism of action responsible for the anti-inflammatory effect is not yet fully elucidated. This stu...

journal_title：British journal of haematology

authors： Hochart H,Jenkins PV,Smith OP,White B

更新日期：2006-04-01 00:00:00

abstract：:Globin-gene mapping of DNA from 13 families with normal Hb A2 beta-thalassaemia (both type 1 and type 2) failed to detect any difference from normal in their globin-gene arrangement. We conclude that deletions such as those responsible for gamma beta-thalassaemia or a 'silent' Hb Lepore are not responsible for this ty...

journal_title：British journal of haematology

authors： Kanavakis E,Metaxotou-Mavromati A,Kattamis C,Aksoy M,Weatherall DJ,Wood WG

更新日期：1982-05-01 00:00:00

abstract：:High-dose chemotherapy with autologous stem cell transplantation (SCT) is widely used as a treatment for systemic AL amyloidosis, but its efficacy has not been proved and it has substantial toxicity in this setting. We report here the outcome of 92 patients evaluated at the UK National Amyloidosis Centre who underwent...

journal_title：British journal of haematology

authors： Goodman HJ,Gillmore JD,Lachmann HJ,Wechalekar AD,Bradwell AR,Hawkins PN

更新日期：2006-08-01 00:00:00

abstract：:Haematological characteristics have been compared in 29 subjects with heterozygous beta0 thalassaemia and in 33 subjects with heterozygous beta+ thalassaemia, identified by the type of sickle cell-beta thalassaemia among close relatives, in a Jamaican Negro population. Total haemoglobin, MCV and MCH were significantly...

journal_title：British journal of haematology

authors： Millard DP,Mason K,Serjeant BE,Serjeant GR

更新日期：1977-06-01 00:00:00

abstract：:A novel principle is proposed for a differential separation of live cells (such as leucocytes) from a main flow. A microfluidic device with planar insulated electrodes as the side walls of the channel was manufactured and tested. An array of insulated vertical conductor wires was inserted along the axis of the channel...

journal_title：British journal of haematology

authors： Popa C,Su B,Vadgama P,Cotter F

更新日期：2007-02-01 00:00:00

abstract：:We present a Ph-positive chronic myeloid leukaemia patient who lost a complete cytogenetic response (CCR) of 23 months duration at the time of detection of a deletion, not previously observed, of chromosomes 9 and 22 sequences flanking the translocation breakpoint on the derivate 9 chromosome. To our knowledge, this i...

journal_title：British journal of haematology

authors： Arranz E,Gil-Fernández JJ,Ramiro S,Blas C,Renedo M,Alegre A,Escudero A,Fernández-Rañada JM

更新日期：2002-06-01 00:00:00

abstract：:Antibodies (Abs) that block factor VIII (FVIII) activity occur in hemophilia A patients treated with FVIII replacement therapy and severely impair treatment. In this work, we designed and synthesized ten peptides whose sequences are found in putative epitopes at the surface of a1 and C2 domains of the FVIII molecule. ...

journal_title：British journal of haematology

authors： Chaves DG,Velloso-Rodrigues C,Moreau V,Nguyen C,Villard S,Belisário AR,Granier C,Santoro MM

更新日期：2008-05-01 00:00:00

abstract：:Patients with aplastic anaemia (AA) frequently develop paroxysmal nocturnal haemoglobinuria (PNH) as a late complication. We investigated the frequency of the development of PNH features including a glycosyl phosphatidylinositol (GPI) anchoring defect in 73 Japanese patients with AA. A deficient expression of CD59 was...

journal_title：British journal of haematology

authors： Azenishi Y,Ueda E,Machii T,Nishimura J,Hirota T,Shibano M,Nakao S,Kinoshita T,Mizoguchi H,Kitani T

更新日期：1999-03-01 00:00:00

abstract：:Hydroxyurea increases fetal haemoglobin in many patients with sickle cell anaemia, but its effectiveness in thalassaemia appears to be less consistent. We describe the response to hydroxyurea in an adult male with homozygous beta thalassaemia, symptomatic paraspinal extramedullary haemopoiesis, bone pain, and progress...

journal_title：British journal of haematology

authors： Saxon BR,Rees D,Olivieri NF

更新日期：1998-06-01 00:00:00

abstract：:The proteasome inhibitor, bortezomib, potentially increases cell sensitivity to chemotherapy. This study was performed to determine the overall response rate (ORR), overall survival (OS), progression-free survival (PFS) and toxicity of CHOP (cyclophosphamide, doxorubicin, vincristine and prednisolone) compared to CHOP...

journal_title：British journal of haematology

authors： Furtado M,Johnson R,Kruger A,Turner D,Rule S

更新日期：2015-01-01 00:00:00

abstract：:B-cell clonality analysis is commonly performed by polymerase chain reaction (PCR) targeting the IGH genes although a high false-negative rate is recognized for germinal centre/post-germinal centre B-cell malignancies, especially follicular lymphoma. We assessed the diagnostic value of BIOMED-2 IGK assays and investig...

journal_title：British journal of haematology

authors： Payne K,Wright P,Grant JW,Huang Y,Hamoudi R,Bacon CM,Du MQ,Liu H

更新日期：2011-10-01 00:00:00

abstract：:We report three cases of platelet dysfunction characterized by defective Ca2+ ionophore-induced platelet aggregation without impaired production of thromboxane A2 (TXA2). The patients had mild to moderate bleeding tendencies, and their platelet aggregation and secretion induced by ADP, collagen, arachidonic acid, stab...

journal_title：British journal of haematology

authors： Fuse I,Higuchi W,Uesugi Y,Aizawa Y

更新日期：2001-03-01 00:00:00

abstract：:High-dose cytosine arabinoside (AraC)-containing regimens have shown the highest antileukaemic efficacy of all currently used regimens in the treatment of acute myeloid leukaemia (AML). This study aimed at increasing the antileukaemic potential of high-dose AraC by raising intracellular levels of AraC triphosphate (Ar...

journal_title：British journal of haematology

authors： Braess J,Wegendt C,Jahns-Streubel G,Kern W,Keye S,Unterhalt M,Schleyer E,Hiddemann W

更新日期：2000-05-01 00:00:00

abstract：:Minimal residual disease (MRD) assessment is a powerful prognostic factor for determining the risk of relapse in childhood acute lymphoblastic leukaemia (ALL). In this Swedish multi-centre study of childhood ALL diagnosed between 2002 and 2006, the MRD levels were analysed in 726 follow-up samples in 228 children usin...

journal_title：British journal of haematology

authors： Thörn I,Forestier E,Botling J,Thuresson B,Wasslavik C,Björklund E,Li A,Lindström-Eriksson E,Malec M,Grönlund E,Torikka K,Heldrup J,Abrahamsson J,Behrendtz M,Söderhäll S,Jacobsson S,Olofsson T,Porwit A,Lönnerholm G,R

更新日期：2011-03-01 00:00:00

abstract：:210 patients, with a history of venous thrombosis, have undergone prothrombotic investigations. In nine cases a consistent deficiency of antithrombin was identified. In five there was a reduction in the plasma antigenic concentration of antithrombin and in a further four cases deficiency was due to the presence of a d...

journal_title：British journal of haematology

authors： Harper PL,Luddington RJ,Daly M,Bruce D,Williamson D,Edgar PF,Perry DJ,Carrell RW

更新日期：1991-03-01 00:00:00

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：:Notwithstanding the improvement in treatment results for paediatric T cell acute lymphoblastic leukaemia (T-ALL) it remains important to understand if genetic aberrations influence therapy response. PTEN tumour suppressor gene inactivation is a frequent event in T-ALL but its effect on patient therapy response is deba...

journal_title：British journal of haematology

authors： Paganin M,Grillo MF,Silvestri D,Scapinello G,Buldini B,Cazzaniga G,Biondi A,Valsecchi MG,Conter V,Te Kronnie G,Basso G

更新日期：2018-09-01 00:00:00

abstract：:Hydroxyurea (HU), an inhibitor of DNA synthesis, can also induce haemoglobinization in certain erythroid cell lines. In this study, we report that intracellular peroxides levels were increased in HU-treated murine erythroleukaemia (MEL) cells and that l-acetyl-N-cysteine (LNAC), a potent reducing reagent, had a signif...

journal_title：British journal of haematology

authors： Nagai T,Tarumoto T,Miyoshi T,Ohmine K,Muroi K,Komatsu N,Sassa S,Ozawa K

更新日期：2003-05-01 00:00:00