Recent advances in the understanding of inherited sideroblastic anaemia.

abstract：:A 16-year-old boy with a bleeding disorder since infancy has a long bleeding time, normal platelet count and morphology and normal plasma factor-VIII activities. His platelets undergo normal shape change and primary aggregation in response to ADP but show defective 5-hydroxytryptamine (5-HT) secretion and aggregation ...

journal_title：British journal of haematology

authors： Hardisty RM,Machin SJ,Nokes TJ,Rink TJ,Smith SW

更新日期：1983-04-01 00:00:00

abstract：:We investigated the origin of bone marrow fibroblasts in three bone marrow transplant recipients with aplastic anaemia and leukaemia who received grafts from HLA-identical siblings of opposite sex. The patients were conditioned for transplantation with high doses of cytotoxic drugs and 300--1000 rads total body irradi...

journal_title：British journal of haematology

authors： Golde DW,Hocking WG,Quan SG,Sparkes RS,Gale RP

更新日期：1980-02-01 00:00:00

abstract：:A serial study of coagulation activation and whole-blood viscosity was performed on 37 patients with local or systemic bacterial infection, malaria, or a viral infection. Thrombocytopenia, without consumption of coagulation factors, was the main feature of benign tertian malaria and viral infection, whereas in septica...

journal_title：British journal of haematology

authors： Richardson SG,Matthews KB,Cruickshank JK,Geddes AM,Stuart J

更新日期：1979-07-01 00:00:00

abstract：:In this study, quiescent bone marrow-derived CD34+ erythroid burst-forming units (BFU-E) were found to be resistant to the inhibitory effects of tumour necrosis factor (TNF)-alpha and -beta as well as interferon (IFN)-alpha, -beta and -gamma, in contrast to those stimulated by a combination of erytrhropoietin (Epo) pl...

journal_title：British journal of haematology

authors： Ratajczak J,Kucia M,Reca R,Zhang J,Machalinski B,Ratajczak MZ

更新日期：2003-10-01 00:00:00

abstract：:This review will focus on the strengths and limitations associated with the current standard of care for primary prevention of ischaemic strokes in children with sickle cell anaemia (SCA) - transcranial Doppler ultrasound (TCD) screening followed by regular blood transfusion therapy when TCD measurement is above a thr...

journal_title：British journal of haematology

authors： Jordan LC,Casella JF,DeBaun MR

更新日期：2012-04-01 00:00:00

abstract：:Follicular lymphoma (FL) constitutes a significant proportion of lymphomas and shows frequent relapses. Beyond conventional chemotherapy, new therapeutic approaches have emerged, focussing on the interplay between lymphoma cells and the microenvironment. Here we report the immunophenotypic investigation of the microen...

journal_title：British journal of haematology

authors： Menter T,Tzankov A,Zucca E,Kimby E,Hultdin M,Sundström C,Beiske K,Cogliatti S,Banz Y,Cathomas G,Karjalainen-Lindsberg ML,Grobholz R,Mazzucchelli L,Sander B,Hawle H,Hayoz S,Dirnhofer S

更新日期：2020-05-01 00:00:00

abstract：:Total knee replacement (TKR) is a safe treatment for alleviating pain and restoring physical function in end-stage arthropathy of the knee. First reports of TKR in haemophiliacs date back to the mid-1970s, however detailed information on long-term outcome is scarce. This study evaluated factors influencing the outcome...

journal_title：British journal of haematology

authors： Solimeno LP,Mancuso ME,Pasta G,Santagostino E,Perfetto S,Mannucci PM

更新日期：2009-04-01 00:00:00

abstract：:In this study we investigated the proliferation of three well-documented MM lines and 10 bone marrow samples from myeloma patients in response to rh-SCF alone and combined with Interleukin-6 (IL-6), IL-3 and IL-3/GM-CSF fusion protein PIXY 321. Neoplastic plasma cells were highly purified (> 90%) by immunomagnetic dep...

journal_title：British journal of haematology

authors： Lemoli RM,Fortuna A,Grande A,Gamberi B,Bonsi L,Fogli M,Amabile M,Cavo M,Ferrari S,Tura S

更新日期：1994-12-01 00:00:00

abstract：:A 33-year-old man presented with hepatosplenomegaly and lymphadenopathy. Bone marrow findings were consistent with Philadelphia-positive accelerated-phase chronic granulocytic leukaemia (CGL). Lymph node histology and immunhistochemistry were diagnostic of T lymphoblastic lymphoma. Molecular studies on lymph node DNA ...

journal_title：British journal of haematology

authors： Jacob A,Rowlands DC,Patton N,Holmes JA

更新日期：1994-10-01 00:00:00

abstract：:Between 1990 and 1999, 36 children with refractory anaemia with excess blasts (RAEB) and RAEB in transformation (RAEBt), not associated with Down's syndrome, were diagnosed in Britain. A total of 31 children received intensive chemotherapy, six of whom proceeded to a bone marrow allograft in first remission, whereas t...

journal_title：British journal of haematology

authors： Webb DK,Passmore SJ,Hann IM,Harrison G,Wheatley K,Chessells JM

更新日期：2002-04-01 00:00:00

abstract：:2-3-Dihydroxybenzoic acid was evaluated as a potentially useful, orally effective iron-chelating drug by performing iron balance studies in patients with beta-thalassaemia major. The administration of this substance at 25 mg/kg/d to five patients for 8 d caused an average increase in iron excretion of 4.5 mg/d. When t...

journal_title：British journal of haematology

authors： Peterson CM,Graziano JH,Grady RW,Jones RL,Vlassara HV,Canale VC,Miller DR,Cerami A

更新日期：1976-08-01 00:00:00

abstract：:A 29-year-old man in remission from acute myeloblastic leukaemia was treated by chemoradiotherapy and transplantation of bone marrow (BMT) collected from his HLA identical brother. Engraftment was documented on D12. Transient acute GVHD (grade II) appeared from D34. No infection complicated the BMT. Nevertheless sever...

journal_title：British journal of haematology

authors： Bierling P,Cordonnier C,Fromont P,Rodet M,Tanzer J,Vernant JP,Bracq C,Duedari N

更新日期：1985-04-01 00:00:00

abstract：:This study was performed to assess the methods which gave maximal recovery of purified CD34/45+ cells from a cord blood specimen and optimal growth of progenitors cultured from the purified cells. Cord blood samples were separated using Percoll gradients (either one (1.080) or two successive (1.080 and 1.068) gradient...

journal_title：British journal of haematology

authors： Charbord P,Newton I,Voillat L,Schaal JP,Herve P

更新日期：1996-09-01 00:00:00

abstract：:Dyskeratosis congenita (DC) is an inherited disorder characterized by skin pigmentation, nail dystrophy and mucosal leucoplakia. In 1995 a Dyskeratosis Congenita Registry was established at the Hammersmith Hospital. In the 46 families recruited, 76/83 patients were male, suggesting that the major form of DC is X-linke...

journal_title：British journal of haematology

authors： Knight S,Vulliamy T,Copplestone A,Gluckman E,Mason P,Dokal I

更新日期：1998-12-01 00:00:00

abstract：:In myeloid malignancies, T-cell and NK function has been shown to deteriorate with transformation from pre-leukaemia to advanced disease. Immune dysfunction in solid tumours has been attributed to abnormal signal transduction, possibly through altered expression of intracellular components of the TCR/CD3 complex (e.g....

journal_title：British journal of haematology

authors： Buggins AG,Hirst WJ,Pagliuca A,Mufti GJ

更新日期：1998-03-01 00:00:00

abstract：:The availability of a preparation of recombinant human erythropoietin (rEp) prompted us to investigate the role of Ep in the regulation of megakaryocytopoiesis in mice, using experimental procedures by which the effects on the mitotic and post-mitotic compartment of megakaryocytes could be evaluated separately. In aga...

journal_title：British journal of haematology

authors： Grossi A,Vannucchi AM,Rafanelli D,Rossi Ferrini P

更新日期：1989-04-01 00:00:00

abstract：:During the administration of recombinant human granulocyte colony-stimulating factor (rhG-CSF) or granulocyte-macrophage CSF (rhGM-CSF) we studied the early and late changes of membrane antigen density on neutrophils. RhG-CSF and rhGM-CSF both caused an early transient reduction in blood neutrophilic granulocyte-conce...

journal_title：British journal of haematology

authors： Hansen PB,Kjaersgaard E,Johnsen HE,Gram J,Pedersen M,Nikolajsen K,Hansen NE

更新日期：1993-09-01 00:00:00

abstract：:The very poor outcome of invasive fungal infections (IFI) in patients with haematological malignancies or recipients of haematopoietic stem cell transplantation is largely attributed to their compromised host defence mechanisms. The restoration or augmentation of immune responses in these patients is now considered as...

journal_title：British journal of haematology

authors： Antachopoulos C,Roilides E

更新日期：2005-06-01 00:00:00

abstract：:Platelet aggregation is commonly used to investigate patients with possible dense granule storage pool deficiency (delta SPD), but recent studies have shown that this investigation is not specific or sensitive for this disorder. We describe a simple one-step technique to detect mepacrine loaded platelets by flow cytom...

journal_title：British journal of haematology

authors： Gordon N,Thom J,Cole C,Baker R

更新日期：1995-01-01 00:00:00

abstract：:Prior studies indicate that neutrophil extracellular traps (NETs) are associated with arterial thromboembolism (ATE) and mortality. We investigated the association between NET formation biomarkers (citrullinated histone H3 [H3Cit], cell-free DNA [cfDNA], and nucleosomes) and the risk of ATE and all-cause mortality in ...

journal_title：British journal of haematology

authors： Grilz E,Mauracher LM,Posch F,Königsbrügge O,Zöchbauer-Müller S,Marosi C,Lang I,Pabinger I,Ay C

更新日期：2019-07-01 00:00:00

abstract：:The plasma concentrations of erythropoietin (Ep), soluble transferrin receptors (sTfRs), iron, total iron binding capacity (TIBC) and ferritin were monitored in five leukaemia patients undergoing autologous bone marrow stem cell transplantation (BMSCT) and in 10 lymphoma and 21 ovarian cancer patients undergoing autol...

journal_title：British journal of haematology

authors： Testa U,Rutella S,Martucci R,Scambia G,D'Onofrio G,Pierelli L,Sica S,Benedetti Panici PL,Menichella G,Foti E,Mastroberardino G,Mancuso S,Leone G,Peschle C

更新日期：1997-03-01 00:00:00

abstract：:Hereditary haemochromatosis (HH) is an autosomal recessive disorder characterized by excessive intestinal iron absorption resulting in increased pathological body iron stores. It is typically associated with homozygosity for the c.845G>A (p.C282Y) mutation in the HFE gene. However, other HFE alterations have been repo...

journal_title：British journal of haematology

authors： Silva B,Martins R,Proença D,Fleming R,Faustino P

更新日期：2012-08-01 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：:To date, there is a lack of long-term safety and efficacy data for iron chelation therapy in transfusion-dependent patients with sickle cell disease (SCD). To evaluate the long-term safety and efficacy of deferasirox (a once-daily oral iron chelator), patients with SCD completing a 1-year, Phase II, randomized, defero...

journal_title：British journal of haematology

authors： Vichinsky E,Bernaudin F,Forni GL,Gardner R,Hassell K,Heeney MM,Inusa B,Kutlar A,Lane P,Mathias L,Porter J,Tebbi C,Wilson F,Griffel L,Deng W,Giannone V,Coates T

更新日期：2011-08-01 00:00:00

abstract：:Three patients with probable congenital erythrocytosis were studied to determine the role of erythropoietin (ESF) in their disease. In addition, haemoglobin function was measured and ESF excretion determined in response to reduction in the haemoglobin concentration. In two cases ESF excretion was clearly elevated abov...

journal_title：British journal of haematology

authors： Whitcomb WH,Peschle C,Moore M,Nitschke R,Adamson JW

更新日期：1980-01-01 00:00:00

abstract：:In this paper we describe the placental transport of [3H]vitamin K1 in pregnant rats during the first 24 h after the oral administration of the vitamin. Vitamin K1 in the fetal livers ranged from 0.13% (3 h) to 2% (24 h) of the values found in the corresponding maternal livers. In spite of the low placental transfer o...

journal_title：British journal of haematology

authors： Hamulyák K,de Boer-van den Berg MA,Thijssen HH,Hemker HC,Vermeer C

更新日期：1987-03-01 00:00:00

abstract：:Based on the morphological appearances of the bone marrow and peripheral blood, 43 patients with dysmyelopoietic syndromes were categorized into four types: refractory anaemia with excess of blasts, chronic myelomonocytic leukaemia, primary acquired sideroblastic anaemia and refractory anaemia with cellular marrow, wi...

journal_title：British journal of haematology

authors： Cazzola M,Barosi G,Berzuini C,Dacco M,Orlandi E,Stefanelli M,Ascari E

更新日期：1982-01-01 00:00:00

abstract：:When monocytic leukaemia line U937 cells were incubated in the presence of HgCl2 there was a rapid increase in tissue factor (TF)-dependent procoagulant activity, reaching a maximum (equivalent to the total TF activity observed when cells had been subjected to a freeze/thaw cycle) after 15 min at 50 microM HgCl2 and a...

journal_title：British journal of haematology

authors： Kaneko H,Kakkar VV,Scully MF

更新日期：1994-05-01 00:00:00

abstract：:We recently identified a human minor histocompatibility (H) antigen, encoded by UDP glycosyltransferase 2 family, polypeptide B17 (UGT2B17), whose immunogenicity results from differential expression in donor and recipient cells as a consequence of a homozygous deletion of the UGT2B17 gene. UGT2B17 is highly expressed ...

journal_title：British journal of haematology

authors： Terakura S,Murata M,Nishida T,Emi N,Akatsuka Y,Riddell SR,Morishima Y,Kodera Y,Naoe T

更新日期：2005-04-01 00:00:00

abstract：:The retinoids: all-transretinoic acid (tretinoin), 13-cis retinoic acid (isotretinoin) and the aromatic retinoids etretinate and acitretin have a preventive and therapeutic effect on chemically-induced tumours. Clinically, retinoids have shown variable effectiveness in therapy and/or prevention of oncological diseases...

journal_title：British journal of haematology

authors： Bollag W

更新日期：1991-10-01 00:00:00