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    20609 results match your criteria British journal of haematology[Journal]

    1 OF 413

    The full spectrum of Castleman disease: 273 patients studied over 20 years.
    Br J Haematol 2017 Nov 16. Epub 2017 Nov 16.
    Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique Hôpitaux de Paris (APHP), Paris, France.
    The spectrum of Castleman disease (CD) has considerably extended since its first description in 1956. Recently, an international collaborative working group has reached consensus on the diagnostic criteria and classification of CD. We herein report 273 patients with lymph node histopathology consistent with CD and investigate the newly established diagnostic criteria. Read More

    Fetal haemoglobin induction in sickle cell disease.
    Br J Haematol 2017 Nov 16. Epub 2017 Nov 16.
    Department of Pediatrics, Division of Hematology/Oncology, Baylor College of Medicine, Houston, TX, USA.
    Fetal haemoglobin (HbF, α2γ2) induction has long been an area of investigation, as it is known to ameliorate the clinical complications of sickle cell disease (SCD). Progress in identifying novel HbF-inducing strategies has been stymied by limited understanding of gamma (γ)-globin regulation. Genome-wide association studies (GWAS) have identified variants in BCL11A and HBS1L-MYB that are associated with HbF levels. Read More

    Interleukin-12 and its procoagulant effect on erythrocytes, platelets and fibrin(ogen): the lesser known side of inflammation.
    Br J Haematol 2017 Nov 16. Epub 2017 Nov 16.
    Department of Physiological Sciences, Stellenbosch University, Stellenbosch, South Africa.
    Inflammation, with its associated inflammatory molecules, is integral to most chronic diseases, including the various cardiovascular diseases. Interleukin 12 (IL12) is one of the inflammatory cytokines that is upregulated during inflammation; however, we know very little about its exact effect on red blood cells (RBCs), platelets and fibrin(ogen). IL12 is an important pleiotropic cytokine in early inflammatory responses and has potent immunomodulatory, antitumour and anti-infection activity. Read More


    Potentially inappropriate medication use in elderly non-Hodgkin lymphoma patients is associated with reduced survival and increased toxicities.
    Br J Haematol 2017 Nov 16. Epub 2017 Nov 16.
    Division of Hematology and Medical Oncology, Laura & Isaac Perlmutter Cancer Center at New York University Langone Medical Center, New York, NY, USA.
    Survival outcomes for elderly lymphoma patients are disproportionally inferior to those of younger patients. We examined medication usage at diagnosis for 171 elderly patients (median age 70 years) with aggressive non-Hodgkin lymphoma treated between 2009 and 2014. At least one potentially inappropriate medication was used in 47% of patients according to the Beers Criteria, 59% experienced treatment delays and/or dose reduction and 65% experienced ≥ grade 3 treatment-related toxicities. Read More

    Enumeration and characterization of circulating multiple myeloma cells in patients with plasma cell disorders.
    Br J Haematol 2017 Nov 5. Epub 2017 Nov 5.
    Janssen Research & Development, LLC, Spring House, PA, USA.
    We have developed an automated assay to enumerate and characterize circulating multiple myeloma cells (CMMC) from peripheral blood of patients with plasma cell disorders. CMMC show expression of genes characteristic of myeloma and fluorescence in situ hybridisation results on CMMC correlated well with bone marrow results. We enumerated CMMC from over 1000 patient samples including separate cohorts of newly diagnosed multiple myeloma and high/intermediate risk smouldering multiple myeloma (SMM) with clinical follow-up data. Read More

    Transplant results in adults with Fanconi anaemia.
    Br J Haematol 2017 Nov 2. Epub 2017 Nov 2.
    Istituto Giannina Gaslini, Genova, Italy.
    The outcomes of adult patients transplanted for Fanconi anaemia (FA) have not been well described. We retrospectively analysed 199 adult patients with FA transplanted between 1991 and 2014. Patients were a median of 16 years of age when diagnosed with FA, and underwent transplantation at a median age of 23 years. Read More

    Quantitative mass spectrometry of human reticulocytes reveal proteome-wide modifications during maturation.
    Br J Haematol 2017 Nov 2. Epub 2017 Nov 2.
    Pillar of Engineering Product Development (EPD), Singapore University of Technology & Design (SUTD), Singapore.
    Erythropoiesis is marked by progressive changes in morphological, biochemical and mechanical properties of erythroid precursors to generate red blood cells (RBC). The earliest enucleated forms derived in this process, known as reticulocytes, are multi-lobular and spherical. As reticulocytes mature, they undergo a series of dynamic cytoskeletal re-arrangements and the expulsion of residual organelles, resulting in highly deformable biconcave RBCs (normocytes). Read More

    What is currently known about the genetics of venous thromboembolism at the dawn of next generation sequencing technologies.
    Br J Haematol 2017 Oct 29. Epub 2017 Oct 29.
    Laboratory of Haematology, La Timone Hospital, Marseille, France.
    Venous thromboembolism (VTE) has a strong genetic component. This review summarizes what is known at the seventeen genes that are now well established to harbour VTE-associated genetic variants. In addition, it discusses additional candidate genes that deserve further validation before being claimed as VTE associated genes. Read More

    Outcomes of adults and children with primary mediastinal B-cell lymphoma treated with dose-adjusted EPOCH-R.
    Br J Haematol 2017 Oct 29. Epub 2017 Oct 29.
    Department of Medicine, Weill Cornell Medicine and New York Presbyterian Hospital, New York, NY, USA.
    Treatment with dose-adjusted EPOCH (etoposide, doxorubicin, cyclophosphamide, vincristine, prednisone) chemotherapy and rituximab (DA-EPOCH-R) has become the standard of care for primary mediastinal B-cell lymphoma (PMBCL) at many institutions despite limited data in the multi-centre setting. We report a large, multi-centre retrospective analysis of children and adults with PMBCL treated with DA-EPOCH-R to characterize outcomes and evaluate prognostic factors. We assessed 156 patients with PMBCL treated with DA-EPOCH-R across 24 academic centres, including 38 children and 118 adults. Read More


    Optimizing the management of hereditary haemochromatosis: the value of MRI R2* quantification to predict and monitor body iron stores.
    Br J Haematol 2017 Oct 30. Epub 2017 Oct 30.
    i3S, Instituto de Investigação e Inovação em Saúde, University of Porto, IBMC, Institute for Molecular and Cell Biology, Porto, Portugal.

    A phase 1 clinical trial evaluating marizomib, pomalidomide and low-dose dexamethasone in relapsed and refractory multiple myeloma (NPI-0052-107): final study results.
    Br J Haematol 2017 Oct 26. Epub 2017 Oct 26.
    Dana Farber Cancer Institute, Boston, MA, USA.
    Marizomib (MRZ) is an irreversible, pan-subunit proteasome inhibitor (PI) in clinical development for relapsed/refractory multiple myeloma (RRMM) and glioma. This study analysed MRZ, pomalidomide (POM) and low-dose dexamethasone (Lo-DEX) [PMD] in RRMM to evaluate safety and determine the maximum tolerated dose (MTD) and/or recommended Phase 2 dose (RP2D). Intravenous MRZ (0·3-0·5 mg/m(2) ) was administered over 2 h on days 1, 4, 8, 11; POM (3-4 mg) on days 1-21; and Lo-DEX (5 or 10 mg) on days 1, 2, 4, 5, 8, 9, 11, 12, 15, 16, 22 and 23 of every 28-day cycle. Read More

    Association between maternal haemoglobin and stillbirth: a cohort study among a multi-ethnic population in England.
    Br J Haematol 2017 Oct 26. Epub 2017 Oct 26.
    National Perinatal Epidemiology Unit (NPEU), Nuffield Department of Population Health, University of Oxford, Oxford, UK.
    The study objectives were to examine the association of maternal haemoglobin with stillbirth and perinatal death in a multi-ethnic population in England. We conducted a retrospective cohort analysis using anonymised maternity data from 14 001 women with singleton pregnancies ≥24 weeks' gestation giving birth between 2013 and 2015 in two hospitals - the Royal Wolverhampton NHS Trust and Guy's and St Thomas' NHS Foundation Trust. Multivariable logistic regression analyses were undertaken to analyse the associations between maternal haemoglobin at first visit and at 28 weeks with stillbirth and perinatal death, adjusting for 11 other risk factors. Read More


    Durable graft-versus-leukaemia effects without donor lymphocyte infusions - results of a phase II study of sequential T-replete allogeneic transplantation for high-risk acute myeloid leukaemia and myelodysplasia.
    Br J Haematol 2017 Oct 26. Epub 2017 Oct 26.
    Centre for Haemato-Oncology, Barts Cancer Institute, Queen Mary University of London, London, UK.
    Allogeneic haematopoietic stem-cell transplantation remains the only curative treatment for relapsed/refractory acute myeloid leukaemia (AML) and high-risk myelodysplasia but has previously been limited to patients who achieve remission before transplant. New sequential approaches employing T-cell depleted transplantation directly after chemotherapy show promise but are burdened by viral infection and require donor lymphocyte infusions (DLI) to augment donor chimerism and graft-versus-leukaemia effects. T-replete transplantation in sequential approaches could reduce both viral infection and DLI usage. Read More


    Donor-derived CD19-targeted T cell infusion induces minimal residual disease-negative remission in relapsed B-cell acute lymphoblastic leukaemia with no response to donor lymphocyte infusions after haploidentical haematopoietic stem cell transplantation.
    Br J Haematol 2017 Nov 26;179(4):598-605. Epub 2017 Oct 26.
    Peking University People's Hospital, Peking University Institute of Haematology, Beijing Key Laboratory of Haematopoietic Stem Cell Transplantation, Collaborative Innovation Centre of Haematology, Peking University, Beijing, China.
    Relapse is a common cause of failure in patients with B-cell acute lymphoblastic leukaemia (B-ALL) after haploidentical haematopoietic stem cell transplantation (haplo-HSCT), and non-responders to donor lymphoblastic infusion after HSCT have a very poor prognosis. Although donor-derived CD19-directed chimeric antigen receptor-modified (CAR) T cells can potentially cure leukaemia, their effectiveness and safety have not been confirmed in relapsed B-ALL cases after haplo-HSCT. Between January 2015 and January 2017, two and four patients each received one and two infusions of CAR T cells from haplo-HSCT donors. Read More

    The cost-effectiveness of immediate treatment or watch and wait with deferred chemotherapy for advanced asymptomatic follicular lymphoma.
    Br J Haematol 2017 Oct 26. Epub 2017 Oct 26.
    University College London, London, UK.
    Recent evidence has shown that immediate treatment with rituximab induction, with and without maintenance, substantially reduces the need for further treatment in patients with advanced asymptomatic follicular lymphoma. This analysis estimates the cost-effectiveness of immediate treatment approaches in comparison to a watch and wait approach from the perspective of the UK National Health Service. A Markov decision model was developed to estimate the cost-effectiveness of treatment strategies in patients with asymptomatic follicular lymphoma. Read More

    Current challenges and opportunities in treating adult patients with Philadelphia-negative acute lymphoblastic leukaemia.
    Br J Haematol 2017 Oct 26. Epub 2017 Oct 26.
    Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, MA, USA.
    Significant advances have been made in recent years in the field of Philadelphia-negative acute lymphoblastic leukaemia (ALL). New insights into the biology and genetics of ALL as well as novel clinical observations and new drugs are changing the way we diagnose, risk-stratify and treat adult patients with ALL. New genetic subtypes and alterations refine risk stratification and uncover new actionable therapeutic targets. Read More

    α-Haemoglobin pool measurement: a useful biomarker for evaluation of β-thalassaemia intermedia? - response to Huang and Li.
    Br J Haematol 2017 Oct 26. Epub 2017 Oct 26.
    Institut National de la Santé et de la Recherche Médicale (Inserm)-U955, équipe 2 : Transfusion et Maladies du Globule Rouge, Institut Mondor de Recherche Biomédicale (IMRB), Université de Paris Est Créteil (UPEC), Créteil, France.

    Effects of vascular endothelial growth factors and their receptors on megakaryocytes and platelets and related diseases.
    Br J Haematol 2017 Oct 26. Epub 2017 Oct 26.
    Department of Experimental Medicine, General Hospital of Shenyang Military Region, Shenyang, Liaoning, China.
    It is well known that vascular endothelial growth factors (VEGFs) and their receptors (vascular endothelial growth factor receptors, VEGFRs) are expressed in different tissues, and VEGF-VEGFR loops regulate a wide range of responses, including metabolic homeostasis, cell proliferation, migration and tubuleogenesis. As ligands, VEGFs act on three structurally related VEGFRs (VEGFR1, VEGFR2 and VEGFR3 [also termed FLT1, KDR and FLT4, respectively]) that deliver downstream signals. Haematopoietic stem cells (HSCs), megakaryocytic cell lines, cultured megakaryocytes (MKs), primary MKs and abnormal MKs express and secrete VEGFs. Read More

    Minimal residual disease in non-Hodgkin lymphoma - current applications and future directions.
    Br J Haematol 2017 Oct 26. Epub 2017 Oct 26.
    Department of Medicine, Brigham and Women's Hospital, Boston, MA, USA.
    Non-Hodgkin Lymphomas (NHLs) are a heterogeneous group of tumours with distinct treatment paradigms, but in all cases the goal of treatment is to maximize quality and duration of remission while minimizing therapy-related toxicity. Identification of persistent disease or relapse is most often the trigger to intensify or re-initiate anti-neoplastic therapy, respectively. In the current era of NHL treatment, this determination is mostly based on imaging and clinical evaluations, tools with imperfect sensitivity and specificity. Read More



    Beneficial effect of exogenous platelet factor 4 for detecting pathogenic heparin-induced thrombocytopenia antibodies.
    Br J Haematol 2017 Oct 19. Epub 2017 Oct 19.
    Department of Haematology-Haemostasis, University Hospital of Tours, Tours, France.
    The laboratory diagnosis of heparin-induced thrombocytopenia (HIT) is based on an enzyme immunoassay combined with a functional test, and serotonin release assay (SRA) is the gold standard for detecting activating HIT antibodies. However, a recent atypical history of HIT prompted us to evaluate whether addition of platelet factor 4 (PF4) during SRA could improve its ability to detect pathogenic HIT antibodies. Using 5B9, a monoclonal antibody to PF4/H with a human Fc fragment, we first defined the optimal PF4 concentration for detecting low amounts of platelet-activating IgG with SRA. Read More

    Realgar nanoparticles versus ATO arsenic compounds induce in vitro and in vivo activity against multiple myeloma.
    Br J Haematol 2017 Oct 19. Epub 2017 Oct 19.
    Cancer Research Institute, Biomedical Research Center SAS, Bratislava, Slovakia.
    Multiple myeloma (MM), a B cell malignancy characterized by clonal proliferation of plasma cells in the bone marrow, remains incurable despite the use of novel and conventional therapies. In this study, we demonstrated MM cell cytotoxicity triggered by realgar (REA; As4 S4 ) nanoparticles (NREA) versus Arsenic trioxide (ATO) against MM cell lines and patient cells. Both NREA and ATO showed in vivo anti-MM activity, resulting in significantly decreased tumour burden. Read More

    How I manage relapse of chronic myeloid leukaemia after stopping tyrosine kinase inhibitor therapy.
    Br J Haematol 2017 Oct 19. Epub 2017 Oct 19.
    Unicancer Center, Institut Bergonié and France Intergroupe des Leucémies Myéloïdes Chroniques (Fi-LMC), Bordeaux, France.
    During the last 10 years, clinical trials formally demonstrated that about 50% of patients with chronic phase (CP) chronic myeloid leukaemia (CML) who achieve and maintain deep molecular responses for a prolonged period of time during treatment with imatinib or new generation tyrosine kinase inhibitors (TKIs) may successfully stop their anti-leukaemic therapy. Based on the accumulated knowledge from abundant clinical trial experience, TKI discontinuation is becoming an important goal to achieve and is about to enter clinical practice. This review focuses on relapse definition, laboratory tests to identify relapse and relapse management after TKI discontinuation. Read More



    How we diagnose and treat systemic mastocytosis in adults.
    Br J Haematol 2017 Oct 19. Epub 2017 Oct 19.
    Department of Hematology and Oncology, Oregon Health and Science University, Portland, OR, USA.
    Rapid advances in the understanding of the molecular biology, data from translational and clinical trials, and retrospective analyses has influenced the diagnosis and treatment of systemic mastocytosis (SM). Many options have existed for the symptomatic management of SM patients, but recent evolution in regards to the molecular underpinnings of this disease and our ability to distinguish clonal mastocytosis from mast cell activation syndrome has changed our treatment paradigm and opened new opportunities for understanding genetic risk, transformation to mast cell leukaemia, and treatment choices. Key to this change has been the discovery of the KIT mutation and the use of next generation sequencing to evaluate for co-existing molecular mutations that may define the disease course. Read More

    Evaluation of a disease risk index for adult patients undergoing umbilical cord blood transplantation for haematological malignancies.
    Br J Haematol 2017 Oct 19. Epub 2017 Oct 19.
    Eurocord, Hôpital Saint Louis, Paris, France.
    A disease risk index (DRI) has been defined for stratifying heterogeneous cohorts of patients undergoing allogeneic haematopoietic stem cell transplantation (HSCT). This index defines 4 distinct groups with different outcomes, dividing patients by disease type and status and considering cytogenetics for acute myeloid leukaemia and myelodysplastic syndromes (MDS). Recently, the DRI has been refined to include rare diseases and improve MDS stratification by blast percentage and response to prior therapy. Read More

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