Molecular characterization of a normal Hb A2 beta-thalassaemia determinant in a Sardinian family.

abstract：:Immuno-alkaline phosphatase staining (by the APAAP technique) has been used to identify promegakaryoblasts in cell smears from 10 cases of leukaemia (three acute leukaemia, seven blast transformations). In all cases promegakaryoblasts were labelled by at least two anti-platelet glycoprotein (gp) antibodies, the highes...

journal_title：British journal of haematology

authors： Erber WN,Breton-Gorius J,Villeval JL,Oscier DG,Bai Y,Mason DY

更新日期：1987-01-01 00:00:00

abstract：:The highly conserved 350-bp major regulatory element HS-40 (or alphaMRE) upstream of the human alpha-globin gene cluster is involved in the regulation of alpha-globin gene expression. The study of alphaMRE differences between human populations and the evolution of alphaMRE sequences in mammals may lead to a better und...

journal_title：British journal of haematology

authors： Harteveld CL,Muglia M,Passarino G,Kielman MF,Bernini LF

更新日期：2002-12-01 00:00:00

abstract：:A phase II A study was conducted to evaluate the safety and efficacy of Givinostat, a novel Histone-Deacetylases inhibitor, in patients with Polycythaemia Vera (PV, n = 12), Essential Thrombocythaemia (ET, n = 1) and Myelofibrosis (n = 16), bearing the JAK2V617F mutation. The study was approved by the local ethics com...

journal_title：British journal of haematology

authors： Rambaldi A,Dellacasa CM,Finazzi G,Carobbio A,Ferrari ML,Guglielmelli P,Gattoni E,Salmoiraghi S,Finazzi MC,Di Tollo S,D'Urzo C,Vannucchi AM,Barosi G,Barbui T

更新日期：2010-08-01 00:00:00

abstract：:Diamond-Blackfan anaemia (DBA) is a rare bone marrow failure syndrome characterised by anaemia, congenital anomalies and cancer predisposition. Although infections are the second leading cause of mortality in non-transplanted patients, immune function is largely unexplored. We identified quantitative deficits in serum...

journal_title：British journal of haematology

authors： Iskander D,Roberts I,Rees C,Szydlo R,Alikian M,Neale M,Harrington Y,Kelleher P,Karadimitris A,de la Fuente J

更新日期：2019-07-01 00:00:00

abstract：:The prognostic role of CD20 expression and Epstein-Barr virus (EBV) positivity in post-transplant lymphoproliferative disease (PTLD) after solid organ transplantation (SOT) in paediatric patients is poorly understood. We retrospectively examined the relationship of CD20 and EBV with the time interval from SOT to PTLD ...

journal_title：British journal of haematology

authors： Orjuela MA,Alobeid B,Liu X,Siebert AL,Kott ER,Addonizio LJ,Morris E,Garvin JH,Lobritto SJ,Cairo MS

更新日期：2011-03-01 00:00:00

abstract：:Acute promyelocytic leukaemia (APL) is a rare subtype of acute myeloid leukaemia. The outcome of paediatric APL has improved substantially over the past 20 years; cure rates above 80% are expected when all-trans retinoic acid (ATRA) is given with anthracycline-based regimens. The presenting features of paediatric APL ...

journal_title：British journal of haematology

authors： Abla O,Ribeiro RC

更新日期：2014-01-01 00:00:00

abstract：:Graft-versus-host disease (GVHD) is a major cause of transplant-related mortality (TRM) after allogeneic haematopoietic stem cell transplantation (HSCT) and presents a challenge in haploidentical HSCT. GVHD may be prevented by ex vivo graft T-cell depletion or in vivo depletion of proliferating lymphocytes. However, b...

journal_title：British journal of haematology

authors： Roy DC,Lachance S,Cohen S,Delisle JS,Kiss T,Sauvageau G,Busque L,Ahmad I,Bernard L,Bambace N,Boumédine RS,Guertin MC,Rezvani K,Mielke S,Perreault C,Roy J

更新日期：2019-09-01 00:00:00

abstract：:Ibrutinib is associated with durable responses in patients with Waldenström macroglobulinaemia (WM). We hypothesized that response depth is predictive of progression-free survival (PFS) in WM patients treated with ibrutinib. Using landmark analyses, we evaluated response depth in two cohorts of WM patients treated wit...

journal_title：British journal of haematology

authors： Castillo JJ,Abeykoon JP,Gustine JN,Zanwar S,Mein K,Flynn CA,Demos MG,Guerrera ML,Kofides A,Liu X,Munshi M,Tsakmaklis N,King R,Yang G,Hunter ZR,Advani RH,Palomba ML,Ansell SM,Gertz MA,Kapoor P,Treon SP

更新日期：2020-11-18 00:00:00

abstract：:Given that donor T cells from a transplant contribute both the desired graft-versus-tumour (GVT) effect and detrimental graft-versus-host disease (GVHD), strategies to separate GVHD and GVT activity are a major clinical goal. We have previously demonstrated that in vivo administration of a recombinant (r)IL-7/HGFβ hyb...

journal_title：British journal of haematology

authors： Hu R,Liu Y,Song Y,Su M,Lu X,Rood D,Lai L

更新日期：2016-11-01 00:00:00

abstract：:Preclinical data suggests anti-lymphoma potential for statins, metformin and cyclooxygenase-2 (COX-2) inhibitors. We performed a retrospective population-based study of all adults aged ≥66 years diagnosed with diffuse large B-cell lymphoma (DLBCL) or transformed lymphoma treated with a rituximab containing regimen, be...

journal_title：British journal of haematology

authors： Smyth L,Blunt DN,Gatov E,Nagamuthu C,Croxford R,Mozessohn L,Cheung MC

更新日期：2020-11-01 00:00:00

abstract：:Recurrent large-scale somatic copy number alterations (SCNAs), and somatic point mutations can be analysed to stratify patients with chronic lymphocytic leukaemia (CLL) into distinct prognostic groups. To investigate the relationship between SCNAs and somatic mutations, we performed whole-exome sequencing and single n...

journal_title：British journal of haematology

authors： Zhou W,Goldin L,Wang M,McMaster ML,Jones K,Burdett L,Chanock SJ,Yeager M,Dean M,Caporaso NE

更新日期：2018-06-01 00:00:00

abstract：:Panobinostat in combination with bortezomib and dexamethasone demonstrated a significant and clinically meaningful progression-free survival benefit compared with placebo, bortezomib and dexamethasone in the phase 3 PANORAMA 1 (Panobinostat Oral in Multiple Myeloma 1) trial. Despite this benefit, patients in the panob...

journal_title：British journal of haematology

authors： San-Miguel JF,Hungria VTM,Yoon SS,Beksac M,Dimopoulos MA,Elghandour A,Jedrzejczak WW,Guenther A,Na Nakorn T,Siritanaratkul N,Schlossman RL,Hou J,Moreau P,Lonial S,Lee JH,Einsele H,Salwender H,Sopala M,Redhu S,Paul S

更新日期：2017-10-01 00:00:00

abstract：:Increased haemoglobin (Hb) A2 levels associated with reduced mean corpuscular volume (MCV) and Hb content per cell (MCH) are the most typical features of heterozygous beta thalassaemia. However, double heterozygotes for alpha and beta thalassaemia may have normal MCV and MCH but Hb A2 always in the carrier range. In t...

journal_title：British journal of haematology

authors： Gasperini D,Cao A,Paderi L,Barella S,Paglietti E,Perseu L,Loi D,Galanello R

更新日期：1993-05-01 00:00:00

abstract：:This exploratory study assessed apoptosis in peripheral blood leucocytes (PBL) from β-thalassaemia patients receiving chronic transfusions and chelation therapy (deferasirox or deferoxamine) at baseline, 1, 6, and 12 months. At baseline, thalassaemic PBLs presented 50% greater levels of Bax (BAX), 75% higher caspase-3...

journal_title：British journal of haematology

authors： Walter PB,Porter J,Evans P,Kwiatkowski JL,Neufeld EJ,Coates T,Giardina PJ,Grady RW,Vichinsky E,Olivieri N,Trachtenberg F,Alberti D,Fung E,Ames B,Higa A,Harmatz P,Thalassemia Clinical Research Network.

更新日期：2013-02-01 00:00:00

abstract：:To determine the effects of L-arginine (L-Arg) supplementation on nitric oxide metabolite (NOx) production, oral L-Arg was given to normal controls, sickle cell disease (SCD) patients at steady state and SCD patients hospitalized with a vaso-occlusive crisis (VOC). L-Arg (0.1 g/kg) increased NOx formation by 18.8 +/- ...

journal_title：British journal of haematology

authors： Morris CR,Kuypers FA,Larkin S,Sweeters N,Simon J,Vichinsky EP,Styles LA

更新日期：2000-11-01 00:00:00

abstract：:Comparative genomic hybridization (CGH) was used to analyse 34 follicular lymphoma (FL) samples. 27 samples showed DNA sequence copy number changes of at least one genomic region (26 samples with at least one gain and nine with at least one loss). Some chromosomes or chromosomal regions were preferentially involved. T...

journal_title：British journal of haematology

authors： Avet-Loiseau H,Vigier M,Moreau A,Mellerin MP,Gaillard F,Harousseau JL,Bataille R,Milpied N

更新日期：1997-04-01 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：:Megakaryocytic (Mk) cell maturation involves polyploidisation, and the number of platelets produced increases with Mk DNA content. Ploidy levels in cultured human MK cells are much lower than those observed in vivo. This study demonstrated that adding the water-soluble vitamin nicotinamide (NIC) to mobilised periphera...

journal_title：British journal of haematology

authors： Giammona LM,Fuhrken PG,Papoutsakis ET,Miller WM

更新日期：2006-11-01 00:00:00

abstract：:Amiodarone has been implicated as a cause of thrombocytopenia but the responsible mechanism is unknown. We performed studies in three patients to characterize the pathogenesis of this complication. No amiodarone-dependent, platelet-reactive antibodies were identified using conventional serological techniques. However,...

journal_title：British journal of haematology

authors： Sahud MA,Caulfield M,Clarke N,Koch R,Bougie D,Aster R

更新日期：2013-10-01 00:00:00

abstract：:Salivary gland mucosa-associated lymphoid tissue (MALT) lymphomas (SGML) are rare, as are data concerning their behaviour. We analysed clinical features at presentation, particularly the association with Sjoegren syndrome (SS) and hepatitis C virus (HCV) infection, and outcome in 33 cases of SGML diagnosed between Mar...

journal_title：British journal of haematology

authors： Ambrosetti A,Zanotti R,Pattaro C,Lenzi L,Chilosi M,Caramaschi P,Arcaini L,Pasini F,Biasi D,Orlandi E,D'Adda M,Lucioni M,Pizzolo G

更新日期：2004-07-01 00:00:00

abstract：:Murine monoclonal antibodies (MoAbs) were produced against the blood group KEL1 glycoprotein (93 kD component) immunopurified from human erythrocytes. One monoclonal antibody, 5A11 (IgGa, kappa), detects by immunoblotting a 93 and 184 kD component from KEL: 1,-2 or KEL: -1,2 red cell membrane preparations, separated b...

journal_title：British journal of haematology

authors： Jaber A,Loirat MJ,Willem C,Bloy C,Cartron JP,Blanchard D

更新日期：1991-10-01 00:00:00

abstract：:The combination of bortezomib and rituximab was evaluated in patients with mantle cell lymphoma (MCL), follicular lymphoma (FL) and Waldenström macroglobulinaemia (WM), in a Phase I and later, a randomized Phase II study. In the randomized study, 42 patients with recurrent/refractory disease received either: bortezomi...

journal_title：British journal of haematology

authors： Agathocleous A,Rohatiner A,Rule S,Hunter H,Kerr JP,Neeson SM,Matthews J,Strauss S,Montoto S,Johnson P,Radford J,Lister A

更新日期：2010-11-01 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：:Seven children (age range 1.8-11 years) with juvenile chronic myelomonocytic leukaemia (JCML) received an allogeneic bone marrow transplantation (BMT), four from an HLA-identical sibling and three from a matched unrelated donor. In the four children transplanted using an HLA-identical sibling, conditioning regimen inc...

journal_title：British journal of haematology

authors： Locatelli F,Pession A,Comoli P,Bonetti F,Giorgiani G,Zecca M,Taibi RM,Mongini ME,Ambroselli F,de Stefano P,Severi F,Paolucci G

更新日期：1996-01-01 00:00:00

abstract：:Two single-nucleotide substitutions in PKLR constituted the molecular basis underlying pyruvate kinase (PK) deficiency in a patient with severe haemolytic anaemia. One novel mutation, IVS5+1G>A, abolished the intron 5 donor splice site. The other mutation, c.1436G>A, altered the intron 10 donor splice site consensus s...

journal_title：British journal of haematology

authors： Wijk R,van Wesel AC,Thomas AA,Rijksen G,van Solinge WW

更新日期：2004-04-01 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：: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：: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：:Megakaryopoiesis was examined in 10 patients (eight females and two males) with cyclic thrombocytopenia (CT) to investigate the underlying pathogenesis. Numbers of CFU-Meg and megakaryocytes and the mean cytoplasmic area (mean area) of megakaryocytes at the peak, nadir, ascent mid phase, and descent mid phase of the p...

journal_title：British journal of haematology

authors： Nagasawa T,Hasegawa Y,Kamoshita M,Ohtani K,Komeno T,Itoh T,Shinagawa A,Kojima H,Ninomiya H,Abe T

更新日期：1995-09-01 00:00:00

abstract：:The main objective of the present study was to determine the role of CD34+ cell subsets in the haemopoietic recovery of children undergoing peripheral blood stem cell transplantation. For this purpose, 38 leukaphereses from 33 children with malignancies mobilized with G-CSF were analysed. Using dual-colour flow cytome...

journal_title：British journal of haematology

authors： Gonzalez-Requejo A,Madero L,Díaz MA,Villa M,García-Escribano C,Balas A,Lillo R,García-Sanchez F,Benito A,Vicario JL

更新日期：1998-04-01 00:00:00