20,923 results match your criteria British journal of haematology[Journal]


Added value of speckle tracking in the evaluation of cardiac function in patients with sickle cell disease.

Br J Haematol 2018 Jun 19. Epub 2018 Jun 19.

Département d'hémato-oncologie, service de Médecine Interne, CHU-Brugmann, Bruxelles, Belgium.

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HAX-1 overexpression in multiple myeloma is associated with poor survival.

Br J Haematol 2018 Jun 19. Epub 2018 Jun 19.

Division of Molecular Toxicology, Institute of Environmental Medicine, Karolinska Institutet, Stockholm, Sweden.

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"Faggot" neutrophils in acute promyelocytic leukaemia with ongoing tretinoin therapy.

Br J Haematol 2018 Jun 19. Epub 2018 Jun 19.

Department of Pathology, Duke University Medical Center, Durham, NC, USA.

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June 2018
1 Read

Augmented NRF2 activation protects adult sickle mice from lethal acute chest syndrome.

Br J Haematol 2018 Jun 19. Epub 2018 Jun 19.

Division of Hematology/Oncology, University of Pittsburgh, Pittsburgh, PA, USA.

Acute chest syndrome (ACS) mortality in sickle cell disease (SCD) rises sharply in young adult patients and mechanism-based prophylaxis is lacking. In SCD, haem oxygenase-1 (HO-1) declines with age and ACS is associated with low HO-1. To test if enhanced HO-1 can reduce ACS mortality, young SCD mice were treated with D3T (3H-1,2-dithiole-3-thione), an activator of nuclear-factor erythroid 2 like 2, which controls HO-1 expression, for 3 months. Read More

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Dyserythropoiesis in the diagnosis of the myelodysplastic syndromes and other myeloid neoplasms: problem areas.

Br J Haematol 2018 Jun 19. Epub 2018 Jun 19.

University of Rennes, CHU Rennes, Inserm, CIC1414, Rennes, France.

An evaluation of the significance of specified dyserythropoietic features in suspected myelodysplastic syndrome (MDS) and acute myeloid leukaemia with erythroid dysplasia was made by means of evaluation of 100 electronic images of bone marrow erythroblasts from each of 20 subjects: 11 with a myeloid neoplasm, six with another condition that could cause erythroid dysplasia and three healthy controls. The evaluation was carried out independently by seven experienced haematologists/haematopathologists who were blinded to the diagnosis. The majority of the dyserythropoietic features listed in the World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues were validated, although karyorrhexis was found to be infrequent and lacking in specificity; multinuclearity and megaloblastosis were more often observed but also lacked specificity. Read More

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Corrigendum.

Authors:

Br J Haematol 2018 Jun;181(6):864

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A British Society for haematology good practice paper on the diagnosis and investigation of patients with mantle cell lymphoma.

Br J Haematol 2018 Jun 8. Epub 2018 Jun 8.

Department of Haematology, Plymouth University Peninsula Schools of Medicine and Dentistry, Plymouth, UK.

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Evaluation of 230 patients with relapsed/refractory deletion 17p chronic lymphocytic leukaemia treated with ibrutinib from 3 clinical trials.

Br J Haematol 2018 Jun 5. Epub 2018 Jun 5.

Chao Family Comprehensive Cancer Center, University of California, Irvine, CA, USA.

Patients with chronic lymphocytic leukaemia/small lymphocytic lymphoma (CLL/SLL) with deletion 17p [del(17p)] have poor outcomes with chemoimmunotherapy. Ibrutinib is indicated for the treatment of CLL/SLL, including del(17p) CLL/SLL, and allows for treatment without chemotherapy. This integrated analysis was performed to evaluate outcomes in 230 patients with relapsed/refractory del(17p) CLL/SLL from three ibrutinib studies. Read More

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June 2018
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Impact of polymorphisms in apoptosis-related genes on the outcome of childhood acute lymphoblastic leukaemia.

Br J Haematol 2018 May 29. Epub 2018 May 29.

Unit of Biological Anthropology, Department of Animal Biology, Plant Biology and Ecology, Faculty of Biosciences, Universitat Autònoma de Barcelona, Bellaterra, Catalonia, Spain.

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May 2018
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Clinical-grade validation of whole genome sequencing reveals robust detection of low-frequency variants and copy number alterations in CLL.

Br J Haematol 2018 May 29. Epub 2018 May 29.

Molecular Diagnostic Centre, Department of Oncology, University of Oxford, Oxford, UK.

The 100 000 Genome Project aims to develop a diagnostics platform by introducing whole genome sequencing (WGS) into clinical practice. Samples from patients with chronic lymphocytic leukaemia were subjected to WGS. WGS detection of single nucleotide variants and insertion/deletions were validated by targeted next generation sequencing showing high concordance (96·3%), also for detection of sub-clonal variants and low-frequency TP53 variants. Read More

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May 2018
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Viral lymphomagenesis: beyond the usual suspects.

Authors:
Jorge J Castillo

Br J Haematol 2018 May 29. Epub 2018 May 29.

Division of Hematologic Malignancies, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.

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Diagnostic markers for CNS lymphoma in blood and cerebrospinal fluid: a systematic review.

Br J Haematol 2018 May 29. Epub 2018 May 29.

University Medical Center Utrecht, Utrecht, The Netherlands.

Diagnosing central nervous system (CNS) lymphoma remains a challenge. Most patients have to undergo brain biopsy to obtain tissue for diagnosis, with associated risks of serious complications. Diagnostic markers in blood or cerebrospinal fluid (CSF) could facilitate early diagnosis with low complication rates. Read More

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Outcome of patients with Myelofibrosis relapsing after allogeneic stem cell transplant: a retrospective study by the Chronic Malignancies Working Party of EBMT.

Br J Haematol 2018 May 29. Epub 2018 May 29.

University Hospital Eppendorf, Hamburg, Germany.

Allogeneic Haematopoietic Stem Cell Transplant (allo-HSCT) remains the only curative approach for Myelofibrosis (MF). Scarce information exists in the literature on the outcome and, indeed, management of those MF patients who relapse following transplant. We hereby report on the management and outcome of 202 patients who relapsed post allo-HSCT for MF. Read More

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Epstein-Barr virus-positive follicular lymphoma.

Br J Haematol 2018 May 29. Epub 2018 May 29.

Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.

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Clinical evolution of lymphomatoid papulosis.

Br J Haematol 2018 May 29. Epub 2018 May 29.

Department of Dermatology, Salford Royal NHS Foundation Trust, Salford, UK.

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Human Pegivirus infection and lymphoma risk and prognosis: a North American study.

Br J Haematol 2018 May 29. Epub 2018 May 29.

Department of Internal Medicine, University of Iowa and Iowa City Veterans Affairs Medical Center, Iowa City, IA, USA.

We evaluated the association of Human Pegivirus (HPgV) viraemia with risk of developing lymphoma, overall and by major subtypes. Because this virus has also been associated with better prognosis in the setting of co-infection with human immunodeficiency virus, we further assessed the association of HPgV with prognosis. We used risk factor data and banked plasma samples from 2094 lymphoma cases newly diagnosed between 2002 and 2009 and 1572 frequency-matched controls. Read More

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How I manage patients with relapsed/refractory diffuse large B cell lymphoma.

Br J Haematol 2018 May 29. Epub 2018 May 29.

Cliniques universitaires UCL Saint-Luc, Brussels, Belgium.

Despite progress in the upfront treatment of diffuse large B cell lymphoma (DLBCL), patients still experience relapses. Salvage chemotherapy followed by autologous stem cell transplantation (ASCT) is the standard second-line treatment for relapsed and refractory (R/R) DLBCL. However, half of the patients will not be eligible for transplantation due to ineffective salvage treatment, and the other half will relapse after ASCT. Read More

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Allogeneic haematopoietic cell transplantation for adult acute myeloid leukaemia in second remission: a retrospective study of the Adult Acute Myeloid Leukaemia Working Group of the Japan Society for Haematopoietic Cell Transplantation (JSHCT).

Br J Haematol 2018 May 29. Epub 2018 May 29.

Division of Clinical Oncology and Haematology, Department of Internal Medicine, Jikei University School of Medicine, Tokyo, Japan.

To evaluate the outcomes and prognostic factors following allogeneic haematopoietic cell transplantation (HCT) for adult acute myeloid leukaemia (AML) in second complete remission (CR2), we retrospectively analysed the Japanese registration data of 1080 adult AML patients in CR2 who had received allogeneic HCT. The probability of overall survival and the cumulative incidence of relapse at 3 years was 66% and 19%, respectively. In multivariate analysis, older age, poor cytogenetics and shorter duration of first complete remission were significantly associated with a higher overall mortality. Read More

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Spectral domain optical coherence tomography interpretation.

Br J Haematol 2018 Jun;181(5):710

Department of Ophthalmology and Visual Sciences, Faculty of Medicine, Dalhousie University, Halifax, Nova Scotia, Canada.

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Effective Response Metric: a novel tool to predict relapse in childhood acute lymphoblastic leukaemia using time-series gene expression profiling.

Br J Haematol 2018 Jun;181(5):653-663

School of Computing, National University of Singapore, Singapore.

Accurate risk assignment in childhood acute lymphoblastic leukaemia is essential to avoid under- or over-treatment. We hypothesized that time-series gene expression profiles (GEPs) of bone marrow samples during remission-induction therapy can measure the response and be used for relapse prediction. We computed the time-series changes from diagnosis to Day 8 of remission-induction, termed Effective Response Metric (ERM-D8) and tested its ability to predict relapse against contemporary risk assignment methods, including National Cancer Institutes (NCI) criteria, genetics and minimal residual disease (MRD). Read More

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June 2018
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Corrigendum.

Authors:

Br J Haematol 2018 Jun 29;181(5):712. Epub 2018 Apr 29.

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Long-term survival after childhood acute lymphoblastic leukaemia: population-based trends in cure and relapse by clinical characteristics.

Br J Haematol 2018 May 29. Epub 2018 May 29.

Clinical and Population Sciences Department, School of Medicine, University of Leeds, Leeds, UK.

'Cure models' offer additional information to traditional epidemiological approaches to assess survival for cancer patients by simultaneously estimating the proportion cured and the survival of those 'uncured'. The proportion cured is a summary of long-term survival while the median survival time of the uncured provides important information on those who are not long-term survivors. Population-based trends in the cure proportion and survival of the uncured for childhood acute lymphoblastic leukaemia (ALL) by clinical prognostic risk factors were estimated using flexible parametric cure models, based on overall survival and event-free survival. Read More

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Will Rogers: actor, humourist … and ALL epidemiologist?

Authors:
Robert Hills

Br J Haematol 2018 May 29. Epub 2018 May 29.

Centre for Trials Research, Cardiff University, Cardiff, UK.

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May 2018
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Elotuzumab monotherapy in patients with smouldering multiple myeloma: a phase 2 study.

Br J Haematol 2018 May 29. Epub 2018 May 29.

Harvard Medical School, Jerome Lipper Multiple Myeloma Center, Dana-Farber Cancer Institute, Boston, MA, USA.

Smouldering multiple myeloma (SMM) is associated with increased risk of progression to multiple myeloma within 2 years, with no approved treatments. Elotuzumab has been shown to promote natural killer (NK) cell stimulation and antibody-dependent cellular cytotoxicity (ADCC) in vitro. CD56 (CD56 /CD16 /CD3 /CD45 ) NK cells represent the primary subset responsible for elotuzumab-induced ADCC. Read More

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How we manage Gaucher Disease in the era of choices.

Br J Haematol 2018 May 29. Epub 2018 May 29.

Gaucher Clinic, Shaare Zedek Medical Centre, Hadassah-Hebrew University Medical School, Jerusalem, Israel.

Treatment of Gaucher Disease (GD) is now beset with the abundance of therapeutic options for an individual patient, making the choice of therapy complex for both expert and non-expert clinicians. The pathogenesis of all disease manifestations is a gene mutation-driven deficiency of glucocerebrosidase, but the clinical expression and response of each of the clinical manifestations to different therapies can be difficult to predict. Enzyme replacement therapy has been available since 1991 and is well-established, with known efficacy and minimal toxicity. Read More

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Detection of platelet autoantibodies to identify immune thrombocytopenia: state of the art.

Br J Haematol 2018 May 29. Epub 2018 May 29.

Immunohaematology Diagnostic Services, Sanquin Diagnostic Services, Amsterdam, the Netherlands.

Immune Thrombocytopenia (ITP) is diagnosed by exclusion of other causes for thrombocytopenia. Reliable detection of platelet autoantibodies would support the clinical diagnosis of ITP and prevent misdiagnosis. We optimized our diagnostic algorithm for suspected ITP using the direct monoclonal antibody immobilization of platelet antigens assay (MAIPA), which evaluates the presence of platelet autoantibodies on the glycoproteins (GP) IIb/IIIa, Ib/IX and V bound on the patient platelets. Read More

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Neonatal leukaemia.

Br J Haematol 2018 May 28. Epub 2018 May 28.

St Mary's Hospital campus of Imperial College London, St Mary's Hospital, London, UK.

Neonatal leukaemia is defined as occurring within the first 28 days of life and most, if not all, cases are congenital. With the exception of Down syndrome-associated transient abnormal myelopoiesis, which is not considered here, neonatal leukaemias are rare. In two-thirds of patients the disease manifests as an acute myeloid leukaemia, frequently with monocytic/monoblastic characteristics. Read More

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May 2018
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Congenital acute myeloid leukaemia with KMT2A rearrangement.

Br J Haematol 2018 May 24. Epub 2018 May 24.

Department of Paediatric Haematology, John Radcliffe Hospital, Oxford, UK.

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Cannibalism by erythroleukaemic blasts.

Br J Haematol 2018 May 24. Epub 2018 May 24.

Department of Haematology Oncology, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Policlinico San Matteo Foundation, Pavia, Italy.

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The diversity of neutrophil inclusion bodies in fulminant sepsis.

Br J Haematol 2018 May 24. Epub 2018 May 24.

Department of Haematology, Ninewells Hospital and Medical School, Dundee, Scotland, UK.

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Transcriptomic rationale for synthetic lethality-targeting ERCC1 and CDKN1A in chronic myelomonocytic leukaemia.

Br J Haematol 2018 May 24. Epub 2018 May 24.

Haematology Department, Hospital Morales Meseguer, IMIB, Murcia, Spain.

Despite the absence of mutations in the DNA repair machinery in myeloid malignancies, the advent of high-throughput sequencing and discovery of splicing and epigenetics defects in chronic myelomonocytic leukaemia (CMML) prompted us to revisit a pathogenic role for genes involved in DNA damage response. We screened for misregulated DNA repair genes by enhanced RNA-sequencing on bone marrow from a discovery cohort of 27 CMML patients and 9 controls. We validated 4 differentially expressed candidates in CMML CD34 bone marrow selected cells and in an independent cohort of 74 CMML patients, mutationally contextualized by targeted sequencing, and assessed their transcriptional behavior in 70 myelodysplastic syndrome, 66 acute myeloid leukaemia and 25 chronic myeloid leukaemia cases. Read More

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Type 1 cryoglobulinaemia leading to gangrene of the toes and ischaemic ulceration.

Br J Haematol 2018 Jun 24;181(6):724. Epub 2018 May 24.

Department of Clinical Haematology, Wycombe Hospital, High Wycombe, UK.

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June 2018
2 Reads

A significant proportion of children of African descent with HbSβ thalassaemia are inaccurately diagnosed based on phenotypic analyses alone.

Br J Haematol 2018 May 24. Epub 2018 May 24.

Vanderbilt-Meharry Center of Excellence in Sickle Cell Disease, Vanderbilt University Medical Center, Nashville, TN, USA.

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Targeted mutation screening of 292 candidate genes in 38 children with inborn haematological cytopenias efficiently identifies novel disease-causing mutations.

Br J Haematol 2018 May 24. Epub 2018 May 24.

St Anna Children's Hospital, Department of Paediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria.

Establishing a precise diagnosis is essential in inborn haematological cytopenias to enable appropriate treatment decisions and avoid secondary organ damage. However, both diversity and phenotypic overlap of distinct disease entities may make the identification of underlying genetic aetiologies by classical Sanger sequencing challenging. Instead of exome sequencing, we established a systematic next generation sequencing-based panel targeting 292 candidate genes and screened 38 consecutive patients for disease-associated mutations. Read More

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Machine learning algorithms for accurate differential diagnosis of lymphocytosis based on cell population data.

Br J Haematol 2018 May 22. Epub 2018 May 22.

Department of Experimental & Health Sciences, Pompeu Fabra University, Barcelona, Spain.

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Safety and efficacy of apixaban for routine thromboprophylaxis in myeloma patients treated with thalidomide- and lenalidomide-containing regimens.

Br J Haematol 2018 May 22. Epub 2018 May 22.

NHS Lothian, Department of Haematology, Western General Hospital, Edinburgh, United Kingdom.

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May 2018
2 Reads

How do we move towards a personalised approach in the treatment of Early Hodgkin lymphoma?

Br J Haematol 2018 May 22. Epub 2018 May 22.

Division of cancer sciences, Faculty of Biology, Medicine and Health, The University of Manchester, NIHR Biomedical Research Centre, Manchester Academic Health Sciences Centre, The Christie NHS Foundation Trust, Manchester, UK.

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Four-year follow-up of a single arm, phase II clinical trial of ibrutinib with rituximab (IR) in patients with relapsed/refractory mantle cell lymphoma (MCL).

Br J Haematol 2018 May 22. Epub 2018 May 22.

Department of Lymphoma and myeloma, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.

Ibrutinib has shown significant activity in patients with relapsed or refractory mantle cell lymphoma (RR-MCL). We report the long-term outcome and safety profile of a single-centre, single arm, open-label, phase 2 study of RR-MCL treated with IR. Overall, the median follow-up time was 47 months (range 1-52 months), median duration on treatment was 16 months (range 1-53 months) and median number of treatment cycles was 17 (range 1-56). Read More

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Patient-reported outcomes of multiple myeloma patients treated with panobinostat after ≥2 lines of therapy based on the international phase 3, randomized, double-blind, placebo-controlled PANORAMA-1 trial.

Br J Haematol 2018 Jun 17;181(5):628-636. Epub 2018 May 17.

Winship Cancer Institute, Emory University School of Medicine, Atlanta, GA, USA.

The phase 3 PANORAMA-1 trial led to regulatory approvals of panobinostat (PAN) in combination with bortezomib (BTZ) and dexamethasone (DEX) for the treatment of multiple myeloma after ≥2 prior regimens, including BTZ and an immunomodulatory drug. Patient-reported outcomes (PROs) were assessed in PANORAMA-1, with data available for 73 patients in the PAN + BTZ + DEX arm and 74 patients in the placebo (PBO) + BTZ + DEX arm. Per the European Organisation for Research and Treatment of Cancer Quality of Life Questionnaire-Core 30 (EORTC QLQ-C30), global health status/quality of life (QoL) scores initially declined with PAN + BTZ + DEX during the first 24 weeks before approaching baseline scores and remaining steady during the next 24 weeks, with no difference between arms at Week 48. Read More

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June 2018
2 Reads