Myelofibrosis biology and contemporary management.

abstract：:Purpura fulminans (PF) is a cutaneous manifestation of a dramatic and deadly syndrome of systemic disseminated intravascular coagulation (DIC). It is characterized by microvascular thrombosis in the dermis followed by perivascular haemorrhage. Since two other related syndromes involve the protein C (PC) system, we und...

journal_title：British journal of haematology

authors： Madden RM,Gill JC,Marlar RA

更新日期：1990-05-01 00:00:00

abstract：:T-cell large granular lymphocyte leukaemia (T-LGL) is a clonal disorder of T cells associated with neutropenia and anaemia. The clinical consequences are recurrent infections and transfusion dependence. The optimum treatment for severely affected patients remains to be defined. Current therapies require long-term admi...

journal_title：British journal of haematology

authors： Sternberg A,Eagleton H,Pillai N,Leyden K,Turner S,Pearson D,Littlewood T,Hatton C

更新日期：2003-02-01 00:00:00

abstract：:Sickle cell anaemia (SS) and sickle cell-haemoglobin C disease (SC) patients exhibit severe red blood cell (RBC) rheological alterations involved in the development of several complications. The contribution of oxidative stress in these haemorheological abnormalities is still unknown. We compared RBC reactive oxygen s...

journal_title：British journal of haematology

authors： Hierso R,Waltz X,Mora P,Romana M,Lemonne N,Connes P,Hardy-Dessources MD

更新日期：2014-08-01 00:00:00

abstract：:Critically ill patients with coronavirus disease 2019 (COVID-19) present with hypoxaemia and are mechanically ventilated to support gas exchange. We performed a retrospective, observational study of blood gas analyses (n = 3518) obtained from patients with COVID-19 to investigate changes in haemoglobin oxygen (Hb-O2 )...

journal_title：British journal of haematology

authors： Vogel DJ,Formenti F,Retter AJ,Vasques F,Camporota L

更新日期：2020-11-01 00:00:00

abstract：:Inhibition of apoptosis (genetically programmed active cell death) by p210 BCR-ABL expression is a mechanism that might contribute to clonal expansion in chronic myeloid leukaemia (CML). Since cell death following exposure to ionizing radiation and many chemotherapeutic agents can occur by the apoptotic pathway, inhib...

journal_title：British journal of haematology

authors： Amos TA,Lewis JL,Grand FH,Gooding RP,Goldman JM,Gordon MY

更新日期：1995-10-01 00:00:00

abstract：:The International Extranodal Lymphoma Study Group (IELSG) promoted this study to determine the inter-observer agreement in the application of the Groupe d' Etude des Lymphomes de l' Adulte (GELA) histological scoring system for evaluating residual disease in post-treatment gastric biopsies of patients with gastric Muc...

journal_title：British journal of haematology

authors： Copie-Bergman C,Wotherspoon AC,Capella C,Motta T,Pedrinis E,Pileri SA,Bertoni F,Conconi A,Zucca E,Ponzoni M,Ferreri AJ

更新日期：2013-01-01 00:00:00

abstract：:Platelet function was evaluated in eight patients with chronic granulocytic leukaemia (CGL), seven Ph1 positive and one Ph1 negative. Seven of the eight patients' platelets had an absence of the second wave of adrenaline induced aggregation on at least one occasion, while five had impaired collagen aggregation. The pl...

journal_title：British journal of haematology

authors： Gerrard JM,Stoddard SF,Shapiro RS,Coccia PF,Ramsay NK,Nesbit ME,Rao GH,Krivit W,White JG

更新日期：1978-12-01 00:00:00

abstract：:The thrombopoietin receptor agonists (THPO-RAs), romiplostim and eltrombopag, are effective and safe in immune thrombocytopenia (ITP). However, the value of their sequential use when no response is achieved or when adverse events occur with one THPO-RA has not been clearly established. Here we retrospectively evaluate...

journal_title：British journal of haematology

authors： González-Porras JR,Mingot-Castellano ME,Andrade MM,Alonso R,Caparrós I,Arratibel MC,Fernández-Fuertes F,Cortti MJ,Pascual C,Sánchez-González B,Bernat S,Fuertes-Palacio MA,Vázquez-Paganini JA,Olivera PE,Alvarez-Román MT,Jarq

更新日期：2015-04-01 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：:Thrombocytopenia is typically observed in patients undergoing cord blood transplantation. We hypothesized that delayed recovery of the platelet count might be caused by defects in the megakaryocytic differentiation pathway of cord blood progenitors. To test this hypothesis, we compared the features of in vitro megakar...

journal_title：British journal of haematology

authors： Miyazaki R,Ogata H,Iguchi T,Sogo S,Kushida T,Ito T,Inaba M,Ikehara S,Kobayashi Y

更新日期：2000-03-01 00:00:00

abstract：:Platelets in an infant with Chediak-Higashi (C-H) syndrome without bleeding manifestations and not in the accelerated phase showed abnormal function consistent with storage pool disorder as shown by abnormal aggregation, decreased storage capacity and release of [14C]5-HT, low endogenous 5-HT, reduced ATP and ADP with...

journal_title：British journal of haematology

authors： Boxer GJ,Holmsen H,Robkin L,Bang NU,Boxer LA,Baehner RL

更新日期：1977-04-01 00:00:00

abstract：:Phenotypic change of blood-type eosinophils to tissue-type eosinophils is induced by various cytokines. We examined the effect of nerve growth factor (NGF) as a candidate for a constitutive cytokine which is able to induce the phenotypic change of eosinophils. The viability of human peripheral blood eosinophils cultur...

journal_title：British journal of haematology

authors： Hamada A,Watanabe N,Ohtomo H,Matsuda H

更新日期：1996-05-01 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：:Imatinib mesylate is useful for facilitating allogeneic stem cell transplantation (allo-SCT) in advanced-phase chronic myelogenous leukaemia (AP-CML) patients. However, although the side-effects of imatinib are usually minor, cardiac morbidity can develop as a latent adverse effect post SCT when a myeloablative SCT is...

journal_title：British journal of haematology

authors： Sohn SK,Kim JG,Kim DH,Lee KB

更新日期：2003-05-01 00:00:00

abstract：:The routine methods for estimating erythrocyte size (volume and surface area) are not generally unbiased. Using geometric models introduces a bias determined by the ways in which real red blood corpuscles depart in size and shape from the simplistic ideal. Employing haematocrit and red corpuscle count to estimate mean...

journal_title：British journal of haematology

authors： Mayhew TM,Mwamengele GL,Self TJ,Travers JP

更新日期：1994-02-01 00:00:00

abstract：:Knowledge of the absolute and relative risk of venous thromboembolism (VTE) in and around pregnancy would be crucial in identifying when to commence and cease thromboprophylaxis in women who would benefit from such intervention. We addressed this hypothesis using a large prospective primary care database from the Unit...

journal_title：British journal of haematology

authors： Sultan AA,West J,Tata LJ,Fleming KM,Nelson-Piercy C,Grainge MJ

更新日期：2012-02-01 00:00:00

abstract：:T-cells from patients with B-cell chronic lymphocytic leukaemia (B-CLL) have abnormal T4/T8 ratios and functions. Previously, we demonstrated that peripheral blood (PB) mononuclear cells from B-CLL patients secrete significant amounts of interleukin 2 (IL2) with an apparent dysregulation of accessory cells controlling...

journal_title：British journal of haematology

authors： Rossi JF,Klein B,Commes T,Jourdan M,Janbon C

更新日期：1988-02-01 00:00:00

abstract：:Routine testing on plasma from a patient due to undergo a coronary artery bypass graft operation revealed a prolonged thrombin clotting time associated with a normal plasma fibrinogen level when this was determined by a method not dependent upon the rate of fibrin formation. Fibrinogen purified from the patient's plas...

journal_title：British journal of haematology

authors： Lane DA,Cuddigan B,VanRoss M,Kakkar VV

更新日期：1980-03-01 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 present study analysed whether autologous peripheral blood stem cell transplantation (PSCT) improves engraftment, quality of life and cost-effectiveness when compared with autologous bone marrow transplantation (ABMT). Relapsing progressive lymphoma patients (n = 204; non-Hodgkin's lymphoma n = 166; Hodgkin's dise...

journal_title：British journal of haematology

authors： Vellenga E,van Agthoven M,Croockewit AJ,Verdonck LF,Wijermans PJ,van Oers MH,Volkers CP,van Imhoff GW,Kingma T,Uyl-de Groot CA,Fibbe WE

更新日期：2001-08-01 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：:Drug-related neutropenia is a common observation in AIDS patients. Haematological growth factors are therefore increasingly used in combination with myelotoxic agents to reduce the risk of infection and to improve the haematological tolerance of these regimens. We report a case of an AIDS patient with Kaposi's sarcoma...

journal_title：British journal of haematology

authors： Hermans P,Gori A,Lemone M,Franchioly P,Clumeck N

更新日期：1994-06-01 00:00:00

abstract：:Allogeneic stem cell transplantation (alloSCT) is a curative procedure for myelofibrosis. Elderly people are mainly affected, limiting the feasibility of myeloablative regimens. The introduction of reduced-intensity conditioning (RIC) made alloSCT feasible for older patients. Nevertheless, the incidence of myelofibros...

journal_title：British journal of haematology

authors： Mannina D,Zabelina T,Wolschke C,Heinzelmann M,Triviai I,Christopeit M,Badbaran A,Bonmann S,von Pein UM,Janson D,Ayuk F,Kröger N

更新日期：2019-08-01 00:00:00

abstract：:The molecular basis accounting for the peculiar clinical and biological features of hairy cell leukaemia (HCL) is currently unknown. Deregulation of cell cycle genes plays a significant role in oncogenesis and there is considerable evidence suggesting that Cdk inhibitors (Ckis) function as tumour suppressors. We and o...

journal_title：British journal of haematology

authors： Chilosi M,Chiarle R,Lestani M,Menestrina F,Montagna L,Ambrosetti A,Prolla G,Pizzolo G,Doglioni C,Piva R,Pagano M,Inghirami G

更新日期：2000-10-01 00:00:00

abstract：:Dickkopf-1 (DKK1), expressed by myeloma cells, suppresses osteoblast function and plays a key role in bone disease in multiple myeloma. BHQ880, a human neutralizing IgG1 anti-DKK1 monoclonal antibody, is being investigated for its impact on multiple myeloma-related bone disease and as an agent with potential anti-myel...

journal_title：British journal of haematology

authors： Iyer SP,Beck JT,Stewart AK,Shah J,Kelly KR,Isaacs R,Bilic S,Sen S,Munshi NC

更新日期：2014-11-01 00:00:00

abstract：:Although the beta thalassaemia trait affects millions of people worldwide, there have been no controlled studies to determine whether it is associated with any clinical disability or abnormal physical signs. To address this question, 402 individuals were studied: 217 with beta thalassaemia trait, of whom 154 were awar...

journal_title：British journal of haematology

authors： Premawardhena A,Arambepola M,Katugaha N,Weatherall DJ

更新日期：2008-05-01 00:00:00

abstract：:The in vitro effects of a range of concentrations of cyclophosphamide, doxorubicin, actinomycin D, cytarabine and methotrexate on neutrophil phagocytosis of C. albicans was studied. The reduction in phagocytic index (PI) was inversely proportional to the dilution of the drug and there was some inhibition of phagocytos...

journal_title：British journal of haematology

authors： Davies JE,Whittaker JA,Khurshid M

更新日期：1976-01-01 00:00:00

abstract：:Three NUP98 chimaeras have previously been reported in T cell acute lymphoblastic leukaemia (T-ALL): NUP98/ADD3, NUP98/CCDC28A, and NUP98/RAP1GDS1. We report a T-ALL with t(11;18)(p15;q12) resulting in a novel NUP98 fusion. Fluorescent in situ hybridisation showed NUP98 and SET binding protein 1(SETBP1) fusion signals...

journal_title：British journal of haematology

authors： Panagopoulos I,Kerndrup G,Carlsen N,Strömbeck B,Isaksson M,Johansson B

更新日期：2007-01-01 00:00:00

abstract：:Use of asparaginase (ASNase) in the treatment of relapsed childhood acute lymphoblastic leukaemia (ALL) is associated with a high rate of hypersensitive reactions. 'Silent' inactivation may additionally reduce treatment intensity. Therefore, PEG-ASNase (Oncaspar), a polyethylene glycol conjugate of the native Escheric...

journal_title：British journal of haematology

authors： Vieira Pinheiro JP,Müller HJ,Schwabe D,Gunkel M,Casimiro da Palma J,Henze G,von Schütz V,Winkelhorst M,Würthwein G,Boos J

更新日期：2001-04-01 00:00:00

abstract：:Myelofibrosis is characterized by splenomegaly and debilitating constitutional symptoms that negatively impact patients' quality of life. ROBUST, a UK, open-label, phase II study, evaluated the safety and efficacy of ruxolitinib in patients with myelofibrosis (N = 48), including intermediate-1 risk patients. The prima...

journal_title：British journal of haematology

authors： Mead AJ,Milojkovic D,Knapper S,Garg M,Chacko J,Farquharson M,Yin J,Ali S,Clark RE,Andrews C,Dawson MK,Harrison C

更新日期：2015-07-01 00:00:00