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    20538 results match your criteria British journal of haematology[Journal]

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    Iron storage in liver, bone marrow and splenic Gaucheroma reflects residual disease in type 1 Gaucher disease patients on treatment.
    Br J Haematol 2017 Sep 14. Epub 2017 Sep 14.
    Department of Internal Medicine, Division of Endocrinology & Metabolism, Academic Medical Centre, University of Amsterdam, Amsterdam, the Netherlands.
    Gaucher disease (GD) is a lysosomal storage disorder characterized by the storage of glycosphingolipids in macrophages. Despite effective therapy, residual disease is present in varying degrees and may be associated with late complications, such as persistent bone or liver disease and increased cancer risk. Gaucher macrophages are capable of storing iron and locations of residual disease may thus be detectable with iron imaging. Read More

    A clinical risk score for pulmonary artery thrombosis during acute chest syndrome in adult patients with sickle cell disease.
    Br J Haematol 2017 Sep 14. Epub 2017 Sep 14.
    Assistance Publique - Hôpitaux de Paris, Hôpitaux Universitaires Henri Mondor, Service de Réanimation Médicale, Créteil.
    Pulmonary artery thrombosis (PAT) is involved in lung vascular dysfunction during acute chest syndrome (ACS) complicating sickle cell disease (SCD). No clinical score is available to identify patients eligible for multi-detector computed tomography (MDCT) angiography during ACS. This retrospective study aimed to develop a risk score for PAT during ACS (PAT-ACS risk score). Read More



    Skeletal and myocardial microvascular blood flow in hydroxycarbamide-treated patients with sickle cell disease.
    Br J Haematol 2017 Sep 7. Epub 2017 Sep 7.
    Knight Cardiovascular Institute, Oregon Health and Science University, Portland, OR, USA.
    In sickle cell disease (SCD), abnormal microvascular function combined with chronic anaemia predisposes patients to perfusion-demand mismatch. We hypothesized that skeletal muscle and myocardial perfusion, normalized to the degree of anaemia, is reduced at basal-state compared to controls, and that this defect is ameliorated by hydroxycarbamide (HC; also termed hydroxyurea) therapy. Twenty-one SCD patients, of whom 15 were treated with HC, and 27 controls underwent contrast-enhanced ultrasound (CEU) perfusion imaging of the forearm as well as the myocardium. Read More


    Congenital neutropenia in the era of genomics: classification, diagnosis, and natural history.
    Br J Haematol 2017 Sep 6. Epub 2017 Sep 6.
    Département de Génétique, AP-HP Hôpital Pitié-Salpêtrière, UPMC Univ Paris 06, Paris, France.
    This review focuses on the classification, diagnosis and natural history of congenital neutropenia (CN). CN encompasses a number of genetic disorders with chronic neutropenia and, for some, affecting other organ systems, such as the pancreas, central nervous system, heart, bone and skin. To date, 24 distinct genes have been associated with CN. Read More

    Results of a multicentre UK-wide retrospective study evaluating the efficacy of brentuximab vedotin in relapsed, refractory classical Hodgkin lymphoma in the transplant naive setting.
    Br J Haematol 2017 Aug 31. Epub 2017 Aug 31.
    Department of Haematology, Churchill Hospital, Oxford University Hospitals NHS Foundation Trust, Oxford, UK.
    Relapsed or refractory classical Hodgkin lymphoma (cHL) is associated with a poor outcome when standard chemotherapy fails. Brentuximab vedotin (BV) is an anti-CD30 monoclonal antibody-drug conjugate licensed for use at relapse after autologous stem cell transplant (ASCT) or following two prior therapies in those unsuitable for ASCT. There are limited data assessing the ability of BV to enable curative SCT. Read More



    Impact of genetic variants on haematopoiesis in patients with thrombocytopenia absent radii (TAR) syndrome.
    Br J Haematol 2017 Aug 31. Epub 2017 Aug 31.
    Institute of Experimental Biomedicine, Chair I, University Hospital Würzburg, Würzburg, Germany.
    Thrombocytopenia absent radii (TAR) syndrome is clearly defined by the combination of radial aplasia and reduced platelet counts. The genetics of TAR syndrome has recently been resolved and comprises a microdeletion on Chromosome 1 including the RBM8A gene and a single nucleotide polymorphism (SNP) either at the 5' untranslated region (5'UTR) or within the first intron of RBM8A. Although phenotypically readily diagnosed after birth, the genetic determination of particular SNPs in TAR syndrome harbours valuable information to evaluate disease severity and treatment decisions. Read More

    A longitudinal evaluation of health-related quality of life in patients with AL amyloidosis: associations with health outcomes over time.
    Br J Haematol 2017 Aug 29. Epub 2017 Aug 29.
    Amyloidosis Center, Boston University School of Medicine, Boston, MA, USA.
    Light chain (AL) amyloidosis is a rare disease associated with significant, irreversible organ dysfunction and high case fatality. An observational study was conducted to assess health-related quality of life (HRQoL) in patients treated for AL amyloidosis between 1994 and 2014 with both high dose melphalan and stem cell transplantation (HDM/SCT) or non-SCT chemotherapy regimens. The SF-36v1(®) Health Survey (SF-36) was administered to assess HRQoL during clinic visits. Read More

    Immune status of patients with haemophilia A before exposure to factor VIII: first results from the HEMFIL study.
    Br J Haematol 2017 Sep 23;178(6):971-978. Epub 2017 Aug 23.
    Faculty of Medicine, Universidade Federal de Minas Gerais, Belo Horizonte, Minas Gerais, Brazil.
    Previous cross-sectional studies showed that some patients with haemophilia A (HA) without inhibitor presented a pro-inflammatory profile during factor VIII (FVIII) replacement therapy. Furthermore, an anti-inflammatory/regulatory state was described in HA patients after inhibitor development. However, no study investigated the levels of these biomarkers before exposure to exogenous FVIII. Read More

    The role of the red blood cell in host defence against falciparum malaria: an expanding repertoire of evolutionary alterations.
    Br J Haematol 2017 Aug 23. Epub 2017 Aug 23.
    MRC International Nutrition Group at Keneba, MRC Unit The Gambia, Banjul, The Gambia.
    The malaria parasite has co-evolved with its human host as each organism struggles for resources and survival. The scars of this war are carried in the human genome in the form of polymorphisms that confer innate resistance to malaria. Clinical, epidemiological and genome-wide association studies have identified multiple polymorphisms in red blood cell (RBC) proteins that attenuate malaria pathogenesis. Read More

    The role of heparin in sepsis: much more than just an anticoagulant.
    Br J Haematol 2017 Aug 18. Epub 2017 Aug 18.
    Department of Intensive Care Unit, the First Affiliated Hospital, China Medical University, Shenyang, Liaoning Province, China.
    Despite progress in antibiotic treatment, mechanical ventilation, fluid resuscitation and blood glucose maintenance, sepsis remains a cause of high mortality in the intensive care unit to date, there are no proven treatment strategies for the routine management of septic patients. The extensive interaction between inflammation and coagulation contributes to the basic pathophysiology of sepsis. Thus, the agents that attenuate the activation of both inflammation and coagulation may improve the outcome in sepsis. Read More

    Outcomes in 370 patients with mantle cell lymphoma treated with ibrutinib: a pooled analysis from three open-label studies.
    Br J Haematol 2017 Aug 18. Epub 2017 Aug 18.
    The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
    Ibrutinib is highly active in treating mantle cell lymphoma (MCL), an aggressive B-cell lymphoma. We pooled data from three ibrutinib studies to explore the impact of baseline patient characteristics on treatment response. Patients with relapsed/refractory MCL (n = 370) treated with ibrutinib had an objective response rate (ORR) of 66% (20% complete response; 46% partial response); median duration of response (DOR), progression-free survival (PFS) and overall survival (OS) were 18·6, 12·8 and 25·0 months, respectively. Read More

    Early-stage Hodgkin lymphoma outcomes after combined modality therapy according to the post-chemotherapy 5-point score: can residual pet-positive disease be cured with radiotherapy alone?
    Br J Haematol 2017 Aug 18. Epub 2017 Aug 18.
    Department of Radiation Oncology, MD Anderson Cancer Center, Houston, TX, USA.
    Early-stage classical Hodgkin lymphoma (HL) patients are evaluated by an end-of-chemotherapy positron emission tomography-computed tomography (eoc-PET-CT) after doxorubicin, bleomycin, vinblastine and dacarbazine (ABVD) and before radiation therapy (RT). We determined freedom from progression (FFP) in patients treated with ABVD and RT according to the eoc-PET-CT 5-point score (5PS). Secondarily, we assessed whether patients with a positive eoc-PET-CT (5PS of 4-5) can be cured with RT alone. Read More

    Diagnosis of CLL revisited: increased specificity by a modified five-marker scoring system including CD200.
    Br J Haematol 2017 Aug 18. Epub 2017 Aug 18.
    Department of Medicine III, University Hospital, LMU Munich, Munich, Germany.
    The modified Matutes score has been the basis for the diagnosis of chronic lymphocytic leukaemia (CLL) by flow cytometry for the past 15 years. To increase the specificity of the current score we systematically evaluated the diagnostic value of established as well as novel markers, such as CD200, in a large cohort of patients with untreated B-cell malignancies (n = 370). Double positivity for CD5 and CD23 was of very high value to differentiate between CLL and non-CLL cases. Read More


    Dexamethasone, rituximab and cyclophosphamide for relapsed and/or refractory and treatment-naïve patients with Waldenstrom macroglobulinemia.
    Br J Haematol 2017 Aug 8. Epub 2017 Aug 8.
    Division of Hematology, Mayo Clinic, Rochester, MN, USA.
    The management of Waldenström macroglobulinaemia (WM) relies predominantly on small trials, one of which has demonstrated activity of dexamethasone, rituximab and cyclophosphamide (DRC) in the frontline setting. We report on the efficacy of DRC, focusing on relapsed/refractory (R/R) patients. Ibrutinib, a recently approved agent in WM demonstrated limited activity in patients with MYD88(WT) genotype. Read More

    Risk of, and survival following, histological transformation in follicular lymphoma in the rituximab era. A retrospective multicentre study by the Spanish GELTAMO group.
    Br J Haematol 2017 Sep 7;178(5):699-708. Epub 2017 Aug 7.
    Hospital Universitario de Salamanca-IBSAL, Salamanca, Spain.
    The diagnostic criteria for follicular lymphoma (FL) transformation vary among the largest series, which commonly exclude histologically-documented transformation (HT) mandatorily. The aims of this retrospective observational multicentre study by the Spanish Grupo Español de Linfoma y Transplante Autólogo de Médula Ósea, which recruited 1734 patients (800 males/934 females; median age 59 years), diagnosed with FL grades 1-3A, were, (i) the cumulative incidence of HT (CI-HT); (ii) risk factors associated with HT; and (iii) the role of treatment and response on survival following transformation (SFT). With a median follow-up of 6·2 years, 106 patients developed HT. Read More

    Whole Genome MBD-seq reveals different CpG methylation patterns in Azacytidine-treated Juvenile Myelomonocytic Leukaemia (JMML) patients.
    Br J Haematol 2017 Aug 2. Epub 2017 Aug 2.
    Department of Paediatric Haematology and Oncology, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) "Bambino Gesù" Children's Hospital, Rome, Italy.



    Activity of lenalidomide in mantle cell lymphoma can be explained by NK cell-mediated cytotoxicity.
    Br J Haematol 2017 Aug 2. Epub 2017 Aug 2.
    Celgene Corporation, Summit, NJ, USA.
    Lenalidomide is an immunomodulatory agent that has demonstrated clinical benefit for patients with relapsed or refractory mantle cell lymphoma (MCL); however, despite this observed clinical activity, the mechanism of action (MOA) of lenalidomide has not been characterized in this setting. We investigated the MOA of lenalidomide in clinical samples from patients enrolled in the CC-5013-MCL-002 trial (NCT00875667) comparing single-agent lenalidomide versus investigator's choice single-agent therapy and validated our findings in pre-clinical models of MCL. Our results revealed a significant increase in natural killer (NK) cells relative to total lymphocytes in lenalidomide responders compared to non-responders that was associated with a trend towards prolonged progression-free survival and overall survival. Read More

    MED12 mutations and NOTCH signalling in chronic lymphocytic leukaemia.
    Br J Haematol 2017 Aug 2. Epub 2017 Aug 2.
    Molecular Therapy in Haematology and Oncology & Department of Translational Oncology, National Centre for Tumour Disease (NCT) and German Cancer Research Centre (DKFZ), Heidelberg, Germany.
    Mutations in the N-terminus of MED12 protein occur at high frequency in uterine leiomyomas and breast fibroepithelial tumours, and are frequently found in chronic lymphocytic leukaemia (CLL). MED12 mutations have been previously linked to aberrant Cyclin C-CDK8 kinase activity, but the exact oncogenic function in CLL is unknown. Here, we characterized MED12 mutations in CLL and identified recurrent mutations in 13 out of 188 CLL patients (6·9%), which clustered in the N-terminus. Read More

    Assessment of the efficacy of a novel tailored vitamin K dosing regimen in lowering the International Normalised Ratio in over-anticoagulated patients: a randomised clinical trial.
    Br J Haematol 2017 Sep 2;178(5):800-809. Epub 2017 Aug 2.
    Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK.
    Current guidelines advocate using fixed-doses of oral vitamin K to reverse excessive anticoagulation in warfarinised patients who are either asymptomatic or have minor bleeds. Over-anticoagulated patients present with a wide range of International Normalised Ratio (INR) values and response to fixed doses of vitamin K varies. Consequently a significant proportion of patients remain outside their target INR after vitamin K administration, making them prone to either haemorrhage or thromboembolism. Read More

    How I manage sickle cell patients with high transcranial doppler results.
    Br J Haematol 2017 Aug 2. Epub 2017 Aug 2.
    Department of Paediatric Haematology, King's College Hospital, London, UK.
    Stroke is one of the most severe complications to affect children with sickle cell anaemia (SCA). Transcranial doppler (TCD) is an accurate and non-invasive method to determine stroke risk. Randomised controlled trials have demonstrated the efficacy of chronic transfusion therapy in stroke prevention based on risk stratification determined by TCD velocities. Read More

    Safety and efficacy of nelarabine in children and young adults with relapsed or refractory T-lineage acute lymphoblastic leukaemia or T-lineage lymphoblastic lymphoma: results of a phase 4 study.
    Br J Haematol 2017 Aug 2. Epub 2017 Aug 2.
    Department of Paediatric Onco-Haematology, APHP, Armand Trousseau Hospital, Paris, France.
    Nelarabine is an antineoplastic agent approved for the treatment of relapsed/refractory T-lineage acute lymphoblastic leukaemia (T-ALL) or T-lineage acute lymphoblastic lymphoma (T-LBL). The purpose of this phase 4, multicentre, single-arm, observational, open-label trial was to provide additional data on the safety and efficacy of nelarabine under licensed conditions of use in children and young adults ≤21 years of age. Patients (N = 28) had a mean ± standard deviation age of 11·5 ± 4·6 years; 71% were male and 61% had a diagnosis of T-ALL. Read More

    Neurotoxic side effects in children with refractory or relapsed T-cell malignancies treated with nelarabine based therapy.
    Br J Haematol 2017 Aug 2. Epub 2017 Aug 2.
    Department of Paediatric Oncology, Haematology, BMT, Charité University Medicine, Berlin, Germany.
    The prognosis in children with refractory or relapsed (r/r) T-cell acute lymphoblastic leukaemia (T-ALL) or lymphoblastic lymphoma (T-LBL) is poor. Nelarabine (Ara-G) has successfully been used as salvage therapy in these children, but has been associated with significant, even fatal, neurotoxicities. We retrospectively analysed 52 patients with r/r T-ALL/T-LBL aged ≤19 years who were treated with Ara-G alone (n = 25) or in combination with cyclophosphamide and etoposide (n = 27). Read More

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