Is refractory anaemia with ring sideroblasts and thrombocytosis (RARS-T) a necessary or useful diagnostic category?

abstract：:We report the characterization of three variant antithrombins with reduced heparin binding as the primary abnormality. Two of these variants, antithrombin Southport (Leu 99 to Val, 2759 C to G) and antithrombin Vienna (Gln 118 to Pro, 5349 A to C) were novel, whereas the third, Pro 41 to Leu, has been previously descr...

journal_title：British journal of haematology

authors： Chowdhury V,Mille B,Olds RJ,Lane DA,Watton J,Barrowcliffe TW,Pabinger I,Woodcock BE,Thein SL

更新日期：1995-03-01 00:00:00

abstract：:Constitutive phosphatidylinositide 3-kinase (PI3K) signalling has been implicated in multiple myeloma (MM) pathophysiology and is regarded as an actionable target for pharmacological intervention. Isoform-specific PI3K inhibition may offer the most focused treatment approach and could result in greater clinical effica...

journal_title：British journal of haematology

authors： Hofmann C,Stühmer T,Schmiedl N,Wetzker R,Mottok A,Rosenwald A,Langer C,Zovko J,Chatterjee M,Einsele H,Bargou RC,Steinbrunn T

更新日期：2014-08-01 00:00:00

abstract：:Interleukin 17A (IL17A), a cytokine involved in allergy, inflammation and osteoclastogenesis, was investigated in multiple myeloma (MM) to assess its role in the osteoclast (OC)-like activity of marrow immature dendritic cells (iDCs). Comparing nine MM patients with control subjects affected by monoclonal gammopathy o...

journal_title：British journal of haematology

authors： Tucci M,Stucci S,Savonarola A,Ciavarella S,Cafforio P,Dammacco F,Silvestris F

更新日期：2013-06-01 00:00:00

abstract：:The close association between translocation of the proto-oncogene bcl-2 and follicular lymphomas has been well established in Caucasian patients and the de-regulation of bcl-2 has been implicated in follicular lymphomagenesis. Similar molecular structural alterations have also been detected in diffuse lymphomas with a...

journal_title：British journal of haematology

authors： Loke SL,Pittaluga S,Srivastava G,Raffeld M,Ho FC

更新日期：1990-09-01 00:00:00

abstract：:Abnormal adhesive interaction between bone marrow stroma and progenitors, one of the causes of unregulated proliferation in chronic myelocytic leukaemia (CML), may be caused by some alterations in adhesion molecules on CML progenitors. We investigated the expression of adhesion molecules (CD44, VLA-5, VLA-4, LFA-1, IC...

journal_title：British journal of haematology

authors： Kawaishi K,Kimura A,Katoh O,Sasaki A,Oguma N,Ihara A,Satow Y

更新日期：1996-05-01 00:00:00

abstract：:Human IgG monoclonal antibodies (mabs) against the Rh D antigen have considerable potential for the prophylaxis of haemolytic disease of the newborn. We have carried out in vitro testing for cross-reactions with tissue components by screening two such mabs against animal tissues and a wide panel of human organs from n...

journal_title：British journal of haematology

authors： Thorpe SJ,Bailey SW

更新日期：1993-02-01 00:00:00

abstract：:This study was undertaken to evaluate the toxicity and pharmacokinetics of a dimethyl sulphoxide (DMSO)-based intravenous formulation of busulphan in the conditioning of 45 patients undergoing allogeneic or autologous stem cell transplantation (SCT). Busulphan was given as a single daily dose. In 15 patients a single ...

journal_title：British journal of haematology

authors： Schuler US,Renner UD,Kroschinsky F,Johne C,Jenke A,Naumann R,Bornhäuser M,Deeg HJ,Ehninger G

更新日期：2001-09-01 00:00:00

abstract：:Idiopathic thrombotic thrombocytopenic purpura (TTP) is characterized by frequent recurrences. Effective screening for relapses will enable intervention prior to overt episodes of TTP. The present study used a modified assay to detect ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 motif, 13) ac...

journal_title：British journal of haematology

authors： Jin M,Casper TC,Cataland SR,Kennedy MS,Lin S,Li YJ,Wu HM

更新日期：2008-05-01 00:00:00

abstract：:We describe a case of congenital dyserythropoietic anaemia (CDA) presenting at week 20 of pregnancy with hydrops fetalis and very severe anaemia. Fetal blood examination showed marked erythroblastosis with morphologic alterations while the basic haematological and biochemical tests were in the normal range. The fetus ...

journal_title：British journal of haematology

authors： Cantù-Rajnoldi A,Zanella A,Conter U,Faccini P,Soligo D,Gornati G,Vegni C,Nicolini U

更新日期：1997-03-01 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：:Primary myelofibrosis (PMF) is a myeloproliferative neoplasm characterized by remodelling of the bone marrow, including progressive myelofibrosis and exaggerated angiogenesis. Advanced PMF frequently shows a full-blown fibre meshwork, which avoids aspiration of cells, and the expression profile of genes related to str...

journal_title：British journal of haematology

authors： Bock O,Muth M,Theophile K,Winter M,Hussein K,Büsche G,Kröger N,Kreipe H

更新日期：2009-09-01 00:00:00

abstract：:Conflicting evidence exists on the epidemiology of type 2 diabetes mellitus (T2DM) among patients with sickle cell disease (SCD). This study measured the prevalence, incidence and clinical outcomes associated with T2DM in a large US population of commercially-insured adults aged ≥20 years with SCD between 2009 and 201...

journal_title：British journal of haematology

authors： Zhou J,Han J,Nutescu EA,Galanter WL,Walton SM,Gordeuk VR,Saraf SL,Calip GS

更新日期：2019-04-01 00:00:00

abstract：:Plasmas having no detectable factor VIII-related antigen but moderate factor VIII coagulant were obtained from two unrelated dogs homozygous for von Willebrand's disease and with the severe clinical expression of the disease. when these plasmas were gel-filtered in a buffer at physiologic ionic strength, the factor VI...

journal_title：British journal of haematology

authors： Benson RE,Johnson GS,Dodds WJ

更新日期：1981-12-01 00:00:00

abstract：:The effect of parenteral administration of IL-11 on gastrointestinal iron absorption was evaluated. A significant increase in the absorption of 59Fe-tagged food iron fed to fasting rats was observed when two subcutaneous injections of IL-11 were given 48 and 24 h prior to testing. Relatively similar increases of 25% w...

journal_title：British journal of haematology

authors： Baynes RD,Cook JD,Keith J

更新日期：1995-09-01 00:00:00

abstract：:18F-fluoro-deoxyglucose (FDG) positron emission tomography (PET) might be a better tool than computerized tomography (CT) in predicting long-term treatment outcome in patients with relapsed chemosensitive lymphoma who are candidates for autologous stem cell transplantation (ASCT). We studied patients with recurrent or...

journal_title：British journal of haematology

authors： Schot B,van Imhoff G,Pruim J,Sluiter W,Vaalburg W,Vellenga E

更新日期：2003-10-01 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：:In advanced chronic myeloid leukaemia patients, STI-571 produces complete haematological response in most cases and cytogenetic response in up to 50%. However, these patients often suffer periods of pancytopenia, which can lead to life-threatening complications, and is probably due to the small number of residual norm...

journal_title：British journal of haematology

authors： Lewalle P,Meuleman N,Verhest A,Bron D,Martiat P

更新日期：2002-07-01 00:00:00

abstract：:A multicentre single-arm study testing the efficacy and toxicity of the oral combination of fludarabine and cyclophosphamide (FC) over 5 d in 75 patients with untreated B cell-chronic lymphocytic leukaemia. Oral FC demonstrated high efficacy with overall (OR) and complete response (CR) rates of 80% and 53%, respective...

journal_title：British journal of haematology

authors： Cazin B,Divine M,Leprêtre S,Travade P,Tournilhac O,Delmer A,Jaubert J,Feugier P,Dreyfus B,Mahé B,Grosbois B,Maloisel F,Eghbali H,Dumontet C,Bénichou J,Guibon O,Leleu X,Leporrier M,Maloum K

更新日期：2008-10-01 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：:Interleukin-12 (IL-12), a heterodimeric cytokine with potent biological activity, was evaluated for effects on proliferation of human immature progenitor cells in vitro. In vitro proliferation of 5-FU-resistant CD34+ (G0) peripheral blood stem cells (PBSC) in response to sequential cytokine stimulation was examined in...

journal_title：British journal of haematology

authors： Grafte-Faure S,Leveque C,Vasse M,Soria C,Vannier JP

更新日期：1999-04-01 00:00:00

abstract：:Despite the importance of adverse event (AE) reporting, AEs are under-reported on clinical trials. We hypothesized that electronic medical record (EMR) data can ascertain laboratory-based AEs more accurately than those ascertained manually. EMR data on 12 AEs for patients enrolled on two Children's Oncology Group (COG...

journal_title：British journal of haematology

authors： Miller TP,Li Y,Getz KD,Dudley J,Burrows E,Pennington J,Ibrahimova A,Fisher BT,Bagatell R,Seif AE,Grundmeier R,Aplenc R

更新日期：2017-04-01 00:00:00

abstract：:A novel long-term cultured interleukin (IL)-3-dependent human myelodysplastic cell line, MDS92, was shown to contain several myeloid-lineage cells such as neutrophils, macrophages, eosinophils, and a small number of megakaryocyte-lineage cells. Therefore this cell line possesses at least bipotential characteristics of...

journal_title：British journal of haematology

authors： Tohyama K,Tohyama Y,Nakayama T,Ueda T,Nakamura T,Yoshida Y

更新日期：1995-12-01 00:00:00

abstract：:The tumour suppressor TP53 (previously termed p53) mediates a pathway that is considered to be one of the most important mechanisms in the maintenance of genomic stability. The function of TP53 can be abrogated by genomic deletion, mutation, or deregulation of upstream and downstream participants in the TP53 pathway. ...

journal_title：British journal of haematology

authors： Cheung KJ,Horsman DE,Gascoyne RD

更新日期：2009-08-01 00:00:00

abstract：:Limited tryptic digestion of native spectrin (Sp) has revealed several variants in hereditary pyropoikilocytosis (HPP) and in a subset of patients with hereditary elliptocytosis (HE). In most cases, tryptic peptide corresponding to the alpha I (N-terminal) 80 kD domain is wholly or partially replaced by smaller fragme...

journal_title：British journal of haematology

authors： Lecomte MC,Gautero H,Garbarz M,Boivin P,Dhermy D

更新日期：1990-11-01 00:00:00

abstract：:Relapsed or refractory (R/R) diffuse large B-cell lymphoma (DLBCL) in those unfit or ineligible for autologous stem cell transplantation is associated with a poor outcome and new treatment approaches are needed. Pixantrone is a novel aza-anthracenedione which is structurally similar to anthracyclines and is licenced i...

journal_title：British journal of haematology

authors： Eyre TA,Linton KM,Rohman P,Kothari J,Cwynarski K,Ardeshna K,Bailey C,Osborne WL,Rowntree C,Eden D,Shankara P,Eyre DW,Jasani P,Chaidos A,Collins GP,Hatton CS

更新日期：2016-06-01 00:00:00

abstract：:Therapy with imatinib mesylate is limited by cellular resistance in chronic myeloid leukaemia (CML). Further, the limited availability of matching stem cell donors or an unfavourable risk profile for allogeneic stem cell transplantation (SCT) reduces the number of therapeutic options in a number of patients. To assess...

journal_title：British journal of haematology

authors： Kreuzer KA,Klühs C,Baskaynak G,Movassaghi K,Dörken B,le Coutre P

更新日期：2004-01-01 00:00:00

abstract：:Refractory anaemia with ring sideroblasts (RARS) is distinguished by hyperplastic inefficient erythropoiesis, aberrant mitochondrial ferritin accumulation and anaemia. Heterozygous mutations in the spliceosome gene SF3B1 are found in a majority of RARS cases. To explore the link between SF3B1 mutations and anaemia, we...

journal_title：British journal of haematology

authors： Conte S,Katayama S,Vesterlund L,Karimi M,Dimitriou M,Jansson M,Mortera-Blanco T,Unneberg P,Papaemmanuil E,Sander B,Skoog T,Campbell P,Walfridsson J,Kere J,Hellström-Lindberg E

更新日期：2015-11-01 00:00:00

abstract：:Multiple Myeloma (MM) is a haematological malignancy characterised by the clonal expansion of plasma cells (PCs) within the bone marrow. Despite advances in therapy, MM remains a largely incurable disease with a median survival of 6 years. In almost all cases, the development of MM is preceded by the benign PC conditi...

journal_title：British journal of haematology

authors： Dutta AK,Hewett DR,Fink JL,Grady JP,Zannettino ACW

更新日期：2017-07-01 00:00:00

abstract：:The reasons why some patients with multiple myeloma (MM) do not develop severe bone loss, or even develop sclerotic bone lesions, remain unclear. In order to answer this question at the cellular and tissue level, we evaluated the histological bone condition of 10 patients with MM who never developed lytic bone lesions...

journal_title：British journal of haematology

authors： Bataille R,Chappard D,Marcelli C,Rossi JF,Dessauw P,Baldet P,Sany J,Alexandre C

更新日期：1990-12-01 00:00:00

abstract：:Therapy for a first episode of venous thromboembolism (VTE) typically includes a vitamin K antagonist, such as warfarin, for 3-6 months at an international normalized ratio (INR) of 2-3. After the cessation of warfarin therapy, unprovoked VTE is associated with a recurrence rate of 5-15% per year. Prolonging initial t...

journal_title：British journal of haematology

authors： Bauer KA

更新日期：2004-10-01 00:00:00