How we manage patients with hereditary haemochromatosis.

abstract：:Severe congenital neutropenia (SCN) is an inherited disorder characterized by severe neutropenia and recurrent infections from an early age, with bone marrow showing a maturational arrest of granulopoiesis at the promyelocyte stage. Since the introduction of G-CSF therapy the prognosis for affected children has improv...

journal_title：British journal of haematology

authors： Ryan M,Will AM,Testa N,Hayworth C,Darbyshire PJ

更新日期：1995-09-01 00:00:00

abstract：:Red cell lifespan has been measured using 51Cr and 59Fe in 19 patients. 59Fe can be used to give results which agree closely with those obtained using 51Cr provided that the plasma 59Fe clearance curve is properly defined and the data analysed correctly. In some patients elution of 51Cr may be more than three times th...

journal_title：British journal of haematology

authors： Ricketts C,Cavill I,Napier JA

更新日期：1977-11-01 00:00:00

abstract：:In acute promyelocytic leukaemia (APL), differentiation therapy can be complicated by the development of a differentiation syndrome (DS). Pulmonary infiltration of differentiating leukaemic cells is a key event in the development of DS. Several mediators have been identified that may promote migration and extravasatio...

journal_title：British journal of haematology

authors： Luesink M,Jansen JH

更新日期：2010-11-01 00:00:00

abstract：:Bone marrow (BM) and peripheral blood (PB) involvement in 10 patients with mantle cell lymphoma (MCL) was analysed by a polymerase chain reaction (PCR)-mediated RNase protection assay. The complementarity determining regions (CDR)-III of all 10 MCLs examined was amplified efficiently with consensus V(H) and J(H) prime...

journal_title：British journal of haematology

authors： Kurokawa T,Kinoshita T,Murate T,Nagasaka T,Kagami Y,Ogura M,Nakamura S,Seto M,Hotta T,Saito H

更新日期：1997-08-01 00:00:00

abstract：:Three identical alpha + thalassemia genes, one of which always carried the Hb J Tongariki mutation, have been observed in Vanuatuans. Despite the fact that at least two of them have arisen by different types of crossover event, the expression of all three haplotypes is identical. ...

journal_title：British journal of haematology

authors： Bowden DK,Pressley L,Higgs DR,Clegg JB,Weatherall DJ

更新日期：1982-06-01 00:00:00

abstract：:Familial aggregation, linkage and case-control studies support the role of germline genes in the aetiology of lymphoid malignancies. To further examine the role of genetic variation underlying susceptibility, we analysed 1536 single nucleotide polymorphisms in 152 genes involved in apoptosis, DNA repair, immune respon...

journal_title：British journal of haematology

authors： Liang XS,Caporaso N,McMaster ML,Ng D,Landgren O,Yeager M,Chanock S,Goldin LR

更新日期：2009-08-01 00:00:00

abstract：:Iron uptake and storage in peripheral blood leucocytes has been studies to test the hypothesis that the basic lesion in idiopathic haemochromatosis resides in a failure of mononuclear phagocytic cells to store iron. In seven fully treated cases of this disease there was no evidence of any abnormality in iron uptake, s...

journal_title：British journal of haematology

authors： Jacobs A,Summers MR

更新日期：1981-12-01 00:00:00

abstract：:Cytogenetic abnormalities (CA), especially of chromosome 13, have been used to identify a subgroup of previously untreated multiple myeloma (MM) patients with very poor prognosis despite high-dose therapy (HDT). We examined the prognostic implications of CA in 1000 MM patients receiving melphalan-based tandem autotran...

journal_title：British journal of haematology

authors： Jacobson J,Barlogie B,Shaughnessy J,Drach J,Tricot G,Fassas A,Zangari M,Giroux D,Crowley J,Hough A,Sawyer J

更新日期：2003-08-01 00:00:00

abstract：:The threat of infection by conventional transfusion-transmitted agents has been essentially eliminated from the blood supply in developed countries, thus focusing attention on the potential risk from emerging infections. Over recent years, actions have been taken to manage a number of such risks to blood safety. These...

journal_title：British journal of haematology

authors： Dodd RY

更新日期：2012-10-01 00:00:00

abstract：:Patients belonging to four families with 'atypical elliptocytosis' have been investigated. Clinical, haematological, erythrokinetic and enzymatic characteristics as well as the effect of splenectomy are discussed. These studies appear to define the fundamental features of a particular disorder or a variety of heredita...

journal_title：British journal of haematology

authors： Torlontano G,Fioritoni G,Salvati AM

更新日期：1979-11-01 00:00:00

abstract：:A systematic analysis of the blast cell population was carried out on samples from 50 patients suffering from blast transformation of chronic granulocytic leukaemia (CGL) (31) and of myelofibrosis (4), acute myelofibrosis (AM) (11) and undifferentiated acute leukaemia (4). Transmission electron microscopy (TEM), used ...

journal_title：British journal of haematology

authors： Polli N,O'Brien M,Tavares de Castro J,Matutes E,San Miguel JF,Catovsky D

更新日期：1985-02-01 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 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：:Mantle cell lymphoma (MCL) is an aggressive non-Hodgkin lymphoma with a poor prognosis following first relapse. We present a subgroup analysis of an open-label phase II trial investigating the efficacy and safety of lenalidomide in patients with relapsed or refractory MCL. Oral lenalidomide 25 mg was self-administered...

journal_title：British journal of haematology

authors： Habermann TM,Lossos IS,Justice G,Vose JM,Wiernik PH,McBride K,Wride K,Ervin-Haynes A,Takeshita K,Pietronigro D,Zeldis JB,Tuscano JM

更新日期：2009-05-01 00:00:00

abstract：:In an attempt to develop a model system for analysing iron metabolism in a relatively homogeneous population of early red cell precursors, the intracellular distribution of 59Fe was examined in Friend murine erythroleukaemic cells after induction of haemoglobin synthesis with dimethylsulphoxide. After incubation of th...

journal_title：British journal of haematology

authors： Peto TE,Rutherford TR,Thompson JL,Weatherall DJ

更新日期：1983-08-01 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：:During the past years, several authors have used labelled leucocytes to detect inflammatory foci. However, before routine use in man. It is necessary to control the viability of labelled cells. Five leucocyte labelling techniques (111In-oxine, 111In-oxine without extraction, 99mTc oxine, pyrophosphate 99mTc, 51Cr) wer...

journal_title：British journal of haematology

authors： Colas-Linhart N,Barbu M,Gougerot MA,Bok B

更新日期：1983-01-01 00:00:00

abstract：:Non-haemolytic transfusion reactions are the most common type of transfusion reaction and include transfusion-related acute lung injury, transfusion-associated circulatory overload, allergic reactions, febrile reactions, post-transfusion purpura and graft-versus- host disease. Although life-threatening anaphylaxis occ...

journal_title：British journal of haematology

authors： Hirayama F

更新日期：2013-02-01 00:00:00

abstract：:A patient presented with haemolytic anaemia and a negative direct antiglobulin test (DAT), and was found to have an IgG antibody with anti-Jka specificity in his serum. His red cells were typed as Jk(a-b+). Later he developed idiopathic thrombocytopenic purpura (ITP), and had a positive DAT due to anti-Jka bound to hi...

journal_title：British journal of haematology

authors： Ganly PS,Laffan MA,Owen I,Hows JM

更新日期：1988-08-01 00:00:00

abstract：:Risk factors for acute graft-versus-host disease (GvHD) remain controversial. We performed uni- and multivariate statistical analyses on a series of 37 patients receiving a non-depleted allogeneic bone marrow transplant from an HLA-identical sibling donor for a haematological malignancy, in order to identify risk fact...

journal_title：British journal of haematology

authors： Bagot M,Mary JY,Heslan M,Kuentz M,Cordonnier C,Vernant JP,Dubertret L,Levy JP

更新日期：1988-12-01 00:00:00

abstract：:Hodgkin's lymphoma (HL) can be cured in most of the patients, but in case of refractory disease or relapse after autologous stem cell transplantation (SCT) the prognosis becomes very poor. In these patients a consensus about the standard approach has not been achieved so far and only allogeneic SCT has shown a long-te...

journal_title：British journal of haematology

authors： Corradini P,Sarina B,Farina L

更新日期：2011-02-01 00:00:00

abstract：:Glucose-6-phosphate dehydrogenase (G6PD) deficient individuals are at increased risk of developing haemolysis following treatment with various antimalarial drugs. Reliable field tests for G6PD deficiency are thus needed in chemotherapy studies and their validity has to be assessed. In two phase II clinical trials on m...

journal_title：British journal of haematology

authors： Meissner PE,Coulibaly B,Mandi G,Mansmann U,Witte S,Schiek W,Müller O,Schirmer RH,Mockenhaupt FP,Bienzle U

更新日期：2005-11-01 00:00:00

abstract：:Therapeutic options for treatment of recurrence of leukaemia after allogeneic bone marrow transplantation (BMT) are limited. A beneficial effect of donor lymphocyte infusions (DLI) has not previously been described in acute myeloid leukaemia (AML) relapse. We report a case of AML with t(8;21), relapsing 3 months after...

journal_title：British journal of haematology

authors： Buzyn-Veil A,Belanger C,Audat F,Hermine O,Bodemer C,Ribrag V,Radford I,Poirel H,Chane C,Valensi F,Macintyre E,Varet B

更新日期：1996-02-01 00:00:00

abstract：:Umbilical cord blood is an alternative stem cell source for patients without matched family donors. In this study, we examined several parameters that have not been studied in detail -- radiation dose, cell dose, age of mice, and maternal and neonatal characteristics of the cord blood donor -- that affect engraftment ...

journal_title：British journal of haematology

authors： Ballen KK,Valinski H,Greiner D,Shultz LD,Becker PS,Hsieh CC,Stewart FM,Quesenberry PJ

更新日期：2001-07-01 00:00:00

abstract：:Chimaerism was studied early (2 weeks to 3 months) during haematopoietic reconstitution after bone marrow transplantation in 18 severe aplastic anaemia patients (acquired SAA: 14 patients; Fanconi anaemia: four patients). Fourteen patients received marrow from an identical sibling donor, one from the phenoidentical fa...

journal_title：British journal of haematology

authors： Socié G,Landman J,Gluckman E,Devergie A,Raynal B,Esperou-Bourdeau H,Brison O

更新日期：1992-03-01 00:00:00

abstract：:Platelets have recently been shown to trigger intrinsic coagulation by two alternative pathways, protect active clotting factors from inactivation by plasma inhibitors and catalyse intrinsic coagulation reactions on the platelet surface to form fibrin. To determine whether these platelet coagulant activities (PCA) mig...

journal_title：British journal of haematology

authors： Walsh PN,Rogers PH,Marder VJ,Gagnatelli G,Escovitz ES,Sherry S

更新日期：1976-03-01 00:00:00

abstract：:Adult survivors of childhood acute lymphoblastic leukaemia (ALL) have a four-fold excess risk of mortality from cardiovascular disease. This cardiovascular risk has not been fully characterized. ALL survivors [n = 784, median age 31·7 years (18·9-59·1)] in the St. Jude Lifetime Cohort Study underwent evaluation for ca...

journal_title：British journal of haematology

authors： Nottage KA,Ness KK,Li C,Srivastava D,Robison LL,Hudson MM

更新日期：2014-05-01 00:00:00

abstract：:In a retrospective study, we examined the association between cytomegalovirus (CMV) infection and non-neutropenic fever immediately following autologous peripheral blood stem cell transplantation for a variety of haematological malignancies and solid tumours. Sixty non-neutropenic febrile episodes (41 in CMV-seroposit...

journal_title：British journal of haematology

authors： Fassas AB,Bolaños-Meade J,Buddharaju LN,Rapoport A,Cottler-Fox M,Chen T,Lovchik JC,Cross A,Tricot G

更新日期：2001-01-01 00:00:00

abstract：:High-dose chemotherapy (HDT) with autologous stem cell transplantation is the standard of care for relapsed/refractory (RR) Hodgkin lymphoma (HL). Given that HDT may cure a sizeable proportion of patients refractory to first salvage, development of newer conditioning regimens remains a priority. We present the results...

journal_title：British journal of haematology

authors： Musso M,Messina G,Di Renzo N,Di Carlo P,Vitolo U,Scalone R,Marcacci G,Scalzulli PR,Moscato T,Matera R,Crescimanno A,Santarone S,Orciuolo E,Merenda A,Pavone V,Pastore D,Donnarumma D,Carella AM,Ciochetto C,Cascavilla

更新日期：2016-01-01 00:00:00

abstract：:Between 1984 and 1992, 21 patients with chronic myeloid leukaemia (CML) in chronic phase (CP) were treated with high-dose chemotherapy (or chemoradiotherapy) followed by autografting with unmanipulated peripheral blood stem cells (PBSC). 12 of these patients survive at a median of 82 months from the time of autografti...

journal_title：British journal of haematology

authors： Hoyle C,Gray R,Goldman J

更新日期：1994-01-01 00:00:00