21,389 results match your criteria British journal of haematology[Journal]


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Br J Haematol 2019 May;185(3):634

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http://dx.doi.org/10.1111/bjh.15926DOI Listing

Detection of a deletion at 22q11 locus involving ZNF280A/ZNF280B/PRAME/GGTLC2 in B-cell malignancies: simply a consequence of an immunoglobulin lambda light chain rearrangement.

Br J Haematol 2019 Apr 15. Epub 2019 Apr 15.

Department of Internal Medicine, Haematology and Oncology, University Hospital Brno and Faculty of Medicine MU, Brno, Czech Republic.

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http://dx.doi.org/10.1111/bjh.15922DOI Listing
April 2019
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Bendamustine for patients with indolent B cell lymphoproliferative malignancies including chronic lymphocytic leukaemia - an updated meta-analysis.

Br J Haematol 2019 Apr 13. Epub 2019 Apr 13.

Tel Aviv University, Tel Aviv.

The question of which chemotherapy induction provides the best results for indolent lymphoma patients is yet unanswered. Different regimens have been compared, none of which has been shown to improve overall survival. The use of bendamustine is growing. Read More

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http://dx.doi.org/10.1111/bjh.15901DOI Listing
April 2019
3 Reads

Impaired cellular and humoral immunity is a feature of Diamond-Blackfan anaemia; experience of 107 unselected cases in the United Kingdom.

Br J Haematol 2019 Apr 13. Epub 2019 Apr 13.

Centre for Haematology, Department of Medicine, Hammersmith Hospital, Imperial College London, London, UK.

Diamond-Blackfan anaemia (DBA) is a rare bone marrow failure syndrome characterised by anaemia, congenital anomalies and cancer predisposition. Although infections are the second leading cause of mortality in non-transplanted patients, immune function is largely unexplored. We identified quantitative deficits in serum immunoglobulins and/or circulating T, natural killer and B lymphocytes in 59 of 107 unselected patients (55·1%) attending our centre over a 7-year period. Read More

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http://dx.doi.org/10.1111/bjh.15915DOI Listing
April 2019
2 Reads

Reinforcing osteopontin as a marker of central nervous system relapse in paediatric B-cell acute lymphoblastic leukaemia: SPP1 splice variant 3 in the spotlight.

Br J Haematol 2019 Apr 13. Epub 2019 Apr 13.

Division of Clinical Research, Research Centre, Instituto Nacional de Câncer-INCA, Rio de Janeiro, RJ, Brazil.

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https://onlinelibrary.wiley.com/doi/abs/10.1111/bjh.15917
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http://dx.doi.org/10.1111/bjh.15917DOI Listing
April 2019
2 Reads

Anthropometric factors and risk of myeloid leukaemias and myelodysplastic syndromes: a prospective study and meta-analysis.

Br J Haematol 2019 Apr 11. Epub 2019 Apr 11.

Epidemiology Research Program, American Cancer Society, Atlanta, GA, USA.

There is insufficient evidence linking excess body weight to risk of myeloid malignancies. We investigated this association using data from the Cancer Prevention Study-II (CPS-II), and a meta-analysis of published cohort studies. Among 152 090 CPS-II participants, 387 acute myeloid leukaemias (AML), 100 chronic myeloid leukaemias (CML) and 170 MDS were identified over 21 years of follow-up. Read More

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http://dx.doi.org/10.1111/bjh.15904DOI Listing
April 2019
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Bone marrow characterization in sickle cell disease: inflammation and stress erythropoiesis lead to suboptimal CD34 recovery.

Br J Haematol 2019 Apr 10. Epub 2019 Apr 10.

Cellular and Molecular Therapeutics Branch, NHLBI/NIDDK, National Institutes of Health, Bethesda, MD, USA.

Stress erythropoiesis and chronic inflammation in subjects with sickle cell disease (SCD) may have an impact on the bone marrow (BM) haematopoietic stem and progenitor cell (HSPC) quality and yield necessary for effective autologous, ex vivo HSPC gene therapy. BM from 19 subjects with SCD and five volunteers without SCD (non-SCD) was collected in different anticoagulants and processed immediately (day 0) or the following day (day 1). Inflammatory, contamination and aggregation markers within the mononuclear layer, and CD34, CD45 and Glycophorin-A (GPA) expression on HSPCs after CD34 selection were analysed by conventional and imaging flow cytometry. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1111/bjh.15902
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http://dx.doi.org/10.1111/bjh.15902DOI Listing
April 2019
6 Reads

Citrullinated histone H3, a biomarker for neutrophil extracellular trap formation, predicts the risk of mortality in patients with cancer.

Br J Haematol 2019 Apr 9. Epub 2019 Apr 9.

Clinical Division of Haematology and Haemostaseology, Department of Medicine I, Medical University of Vienna, Vienna, Austria.

Prior studies indicate that neutrophil extracellular traps (NETs) are associated with arterial thromboembolism (ATE) and mortality. We investigated the association between NET formation biomarkers (citrullinated histone H3 [H3Cit], cell-free DNA [cfDNA], and nucleosomes) and the risk of ATE and all-cause mortality in patients with cancer. In this prospective cohort study, H3Cit, cfDNA and nucleosome levels were determined at study inclusion, and patients with newly diagnosed cancer or progressive disease after remission were followed for 2 years for ATE and death. Read More

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http://dx.doi.org/10.1111/bjh.15906DOI Listing
April 2019
2 Reads

Prognostic significance of mutation profile at diagnosis and mutation persistence during disease remission in adult acute myeloid leukaemia patients.

Br J Haematol 2019 Apr 9. Epub 2019 Apr 9.

Department of Internal Medicine - Hematology and Oncology, University Hospital Brno, Brno, Czech Republic.

In this multi-centre study, we analysed the prognostic impact of mutations in 19 genes associated with myeloid malignancies in 258 newly diagnosed acute myeloid leukaemia patients (aged 19-70 years) undergoing intensive therapy. We identified five patient groups with different prognostic risks and different benefits from allogeneic hematopoietic stem cell transplantation (alloHSCT) within the intermediate cytogenetic risk group patients (n = 184). The most adverse prognosis was observed in patients with DNMT3A and FLT3-ITD co-mutation, whose survival could be significantly improved with alloHSCT. Read More

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http://dx.doi.org/10.1111/bjh.15916DOI Listing
April 2019
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Importance of sequential analysis of TP53 variation in patients with Waldenström Macroglobulinaemia.

Br J Haematol 2019 Apr 9. Epub 2019 Apr 9.

Molecular Pathology, Royal Bournemouth Hospital, Bournemouth, UK.

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http://dx.doi.org/10.1111/bjh.15909DOI Listing

Knockout of von Willebrand factor in Zebrafish by CRISPR/Cas9 mutagenesis.

Br J Haematol 2019 Apr 9. Epub 2019 Apr 9.

Department of Biological Sciences, University of North Texas, Denton, TX, USA.

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https://onlinelibrary.wiley.com/doi/abs/10.1111/bjh.15910
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http://dx.doi.org/10.1111/bjh.15910DOI Listing
April 2019
3 Reads

Bortezomib reinduction chemotherapy in high-risk ALL in first relapse: a report from the Children's Oncology Group.

Br J Haematol 2019 Apr 7. Epub 2019 Apr 7.

Department of Pediatrics and the Center for Childhood Cancer Research, Children's Hospital of Philadelphia, Philadelphia, PA, USA.

While survival in paediatric acute lymphoblastic leukaemia (ALL) is excellent, survival following relapse is poor. Previous studies suggest proteasome inhibition with chemotherapy improves relapse ALL response rates. This phase 2 Children's Oncology Group study tested the hypothesis that adding the proteasome inhibitor bortezomib to chemotherapy increases complete response rates (CR2). Read More

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http://dx.doi.org/10.1111/bjh.15919DOI Listing
April 2019
2 Reads

Chronic lymphocytic leukaemia: the role of T cells in a B cell disease.

Br J Haematol 2019 Apr 3. Epub 2019 Apr 3.

Section of Haematology, Division of Cancer and Genetics, Cardiff University School of Medicine, Cardiff, UK.

Chronic lymphocytic leukaemia (CLL) has long been thought to be an immunosuppressive disease and abnormalities in T-cell subset distribution and function have been observed in many studies. However, the role of T cells (if any) in disease progression remains unclear and has not been directly studied. This has changed with the advent of new therapies, such as chimeric antigen receptor-T cells, which actively use retargeted patient-derived T cells as "living drugs" for CLL. Read More

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http://dx.doi.org/10.1111/bjh.15918DOI Listing
April 2019
1 Read

Complication rates of central venous access devices in patients with inherited bleeding disorders in Australia.

Br J Haematol 2019 Apr 2. Epub 2019 Apr 2.

Australian Centre for Blood Diseases, Monash University Alfred Hospital, Melbourne, VIC, Australia.

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http://dx.doi.org/10.1111/bjh.15914DOI Listing
April 2019
1 Read

Outcomes of second allogeneic haematopoietic stem cell transplantation in patients with relapse of myelodysplastic syndrome.

Br J Haematol 2019 Apr 2. Epub 2019 Apr 2.

Department of Haematology, Kanazawa University Hospital, Kanazawa, Japan.

Though second allogenic haematopoietic stem cell transplantation (HSCT) is considered a curative treatment option after myelodysplastic syndrome (MDS) relapse, scant epidemiological data are available. We investigated the outcomes and prognostic factors of second allogenic HSCT in 99 patients with MDS who relapsed after the first HSCT. The median age was 53 years (interquartile; 45-59) and 57 patients (57·6%) were male. Read More

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http://dx.doi.org/10.1111/bjh.15898DOI Listing
April 2019
2 Reads

Identification of the first large intronic deletion responsible of type I antithrombin deficiency not detected by routine molecular diagnostic methods.

Br J Haematol 2019 Apr 2. Epub 2019 Apr 2.

Servicio de Hematología y Oncología Médica, Hospital Universitario Morales Meseguer, Centro Regional de Hemodonación, Universidad de Murcia, IMIB-Arrixaca, Murcia, CIBERER, Murcia, Spain.

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http://dx.doi.org/10.1111/bjh.15913DOI Listing
April 2019
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Venetoclax is an option in B-cell prolymphocytic leukaemia following progression on B-cell receptor pathway inhibitors.

Br J Haematol 2019 Apr 2. Epub 2019 Apr 2.

Department of Haematology, Rotherham Foundation Trust, Rotherham, UK.

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http://dx.doi.org/10.1111/bjh.15912DOI Listing
April 2019
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The links of hepcidin and erythropoietin in the interplay of inflammation and iron deficiency in a large observational study of rheumatoid arthritis.

Br J Haematol 2019 Apr 2. Epub 2019 Apr 2.

Department of Rheumatology, Immunology and Allergology, Inselspital, Bern University Hospital, Bern, Switzerland.

Anaemia affects quality of life and radiographic outcome in rheumatoid arthritis (RA). In a cross-sectional study with 779 patients, we assessed the prognostic potential of the major haematopoietic regulators, hepcidin and erythropoietin, comparing their serum concentrations with respect to different anaemia types, inflammatory activity, anti-cytokine-specific treatment effects and iron deficiency (ID) indices. The results showed that clinical disease activity was more closely associated with haemoglobin levels than with anti-tumour necrosis factor-alpha or interleukin 6 receptor effects. Read More

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http://dx.doi.org/10.1111/bjh.15895DOI Listing
April 2019
1 Read

Evaluation of the impact of cachexia on clinical outcomes in aggressive lymphoma.

Br J Haematol 2019 Apr 2. Epub 2019 Apr 2.

Rush University Medical Center, Chicago, IL, USA.

Cancer cachexia is a state of involuntary weight loss and altered body composition triggered by an underlying malignancy. We sought to correlate measures of cachexia with clinical outcomes in aggressive lymphomas and to identify biological pathways involved in the cachexia phenotype for possible druggable targets. Radiographic measures of cachexia were collected in a retrospective cohort of 109 patients with aggressive B-cell lymphoma and followed for clinical outcome. Read More

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http://dx.doi.org/10.1111/bjh.15889DOI Listing
April 2019
3 Reads

Leucocytosis-induced plasma hyperkalaemia in samples conveyed by a pneumatic transport system: tips and tricks.

Br J Haematol 2019 Apr 1. Epub 2019 Apr 1.

Section of Clinical Biochemistry, University of Verona, Verona, Italy.

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http://dx.doi.org/10.1111/bjh.15908DOI Listing
April 2019
2 Reads

Romidepsin and total skin electron beam therapy in advanced stage mycosis fungoides and Sézary syndrome.

Br J Haematol 2019 Apr 1. Epub 2019 Apr 1.

Department of Surgery, City of Hope National Medical Center, Duarte, CA, USA.

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http://dx.doi.org/10.1111/bjh.15905DOI Listing
April 2019
3 Reads

High prevalence of altered sudomotor function in homozygous sickle cell patients: influence of age and anaemia.

Br J Haematol 2019 Apr 1. Epub 2019 Apr 1.

Service d'Explorations Fonctionnelles Multidisciplinaires, Assistance Publique - Hôpitaux de Paris (AP-HP), Hôpital Tenon, Paris, France.

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http://dx.doi.org/10.1111/bjh.15892DOI Listing
April 2019
3 Reads

Systemic Notch downregulation promotes Kras -induced myeloproliferative neoplasm.

Br J Haematol 2019 Mar 31. Epub 2019 Mar 31.

McArdle Laboratory for Cancer Research, University of Wisconsin-Madison, Madison, WI, USA.

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http://dx.doi.org/10.1111/bjh.15893DOI Listing

Novel ADA2 mutation presenting with neutropenia, lymphopenia and bone marrow failure in patients with deficiency in adenosine deaminase 2 (DADA2).

Br J Haematol 2019 Mar 28. Epub 2019 Mar 28.

Department of Immunological Medicine, School of Immunology and Microbial Sciences, Faculty of Life Sciences and Medicine, School of medicine, King's College London, King's Health Partners, King's College Hospital NHS Foundation Trust, London, UK.

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http://dx.doi.org/10.1111/bjh.15896DOI Listing
March 2019
2 Reads

Routine sequencing in CLL has prognostic implications and provides new insight into pathogenesis and targeted treatments.

Br J Haematol 2019 Mar 28. Epub 2019 Mar 28.

Department of Leukemia, the University of Texas MD Anderson Cancer Center, Houston, TX, USA.

Chronic lymphocytic leukaemia (CLL) is a genetically heterogeneous disease characterised by genomic alterations and gene mutations that may portend worse survival or resistance to treatments. A total of 680 blood or bone marrow samples underwent targeted sequencing of 29 genes previously identified as being mutated in CLL, which were correlated to known prognostic clinical characteristics. Overall, 400 (59%) patients were treatment-naïve (TN) and 280 (41%) were relapsed/refractory (R/R). Read More

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http://dx.doi.org/10.1111/bjh.15877DOI Listing
March 2019
2 Reads
4.711 Impact Factor

Quantitative sensory testing in children with sickle cell disease: additional insights and future possibilities.

Br J Haematol 2019 Mar 28. Epub 2019 Mar 28.

Nemours Center for Cancer and Blood Disorders, Nemours/Alfred I duPont Hospital for Children, Wilmington, DE, USA.

Quantitative sensory testing (QST) is used in a variety of pain disorders to characterize pain and predict prognosis and response to specific therapies. In this study, we aimed to confirm results in the literature documenting altered QST thresholds in sickle cell disease (SCD) and assess the test-retest reliability of results over time. Fifty-seven SCD and 60 control subjects aged 8-20 years underwent heat and cold detection and pain threshold testing using a Medoc TSAII. Read More

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http://dx.doi.org/10.1111/bjh.15876DOI Listing
March 2019
1 Read

Plasma histamine elevation in a large cohort of sickle cell disease patients.

Br J Haematol 2019 Mar 28. Epub 2019 Mar 28.

Department of Internal Medicine, Tenon Hospital, AP-HP, Paris VI University, Paris, France.

The role of mast cells has been questioned in sickle cell disease (SCD). We performed a prospective study evaluating plasma histamine and tryptase levels in a cohort of paediatric and adult patients, in steady state (n = 132) and during vaso-occlusive crisis (VOC) (n = 121). Histamine level was elevated in 18% of patients in steady state and in 61% during VOC. Read More

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http://dx.doi.org/10.1111/bjh.15900DOI Listing
March 2019
2 Reads

The role of plasma exchange in the management of autoimmune disorders.

Br J Haematol 2019 Mar 28. Epub 2019 Mar 28.

Rheumatology Unit, Department of Medicine, Padova University Hospital, Padova, Italy.

Therapeutic plasma exchange (TPE) has been mainly used in the treatment of autoimmune diseases. The main mechanisms of action of TPE include the removal of circulating autoantibodies, immune complexes, complement components, cytokines and adhesion molecules, along with sensitization of antibody-producing cells to immunosuppressant agents. TPE is useful in autoimmune haematological, renal, rheumatic and neurological diseases, and is recommended for acute disorders, together with relapsed or worsened chronic diseases that are often unresponsive to conventional treatments. Read More

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http://dx.doi.org/10.1111/bjh.15903DOI Listing
March 2019
1 Read

Adult hypodiploid B-acute lymphoblastic leukaemia and stem cell transplantation.

Authors:
Peter H Wiernik

Br J Haematol 2019 Mar 28. Epub 2019 Mar 28.

Cancer Research Foundation of New York, Chappaqua, NY, USA.

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http://dx.doi.org/10.1111/bjh.15882DOI Listing
March 2019
1 Read

Curative potential of chemoimmunotherapy in diffuse large B cell lymphoma with hepatic and/or renal dysfunction.

Br J Haematol 2019 Mar 28. Epub 2019 Mar 28.

Department of Hematologic Oncology and Blood Disorders, Levine Cancer Institute/Atrium Health, Charlotte, NC, USA.

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http://dx.doi.org/10.1111/bjh.15897DOI Listing
March 2019
1 Read

Adoptive donor immunity protects against resolved hepatitis B virus reactivation after allogeneic haematopoietic stem cell transplantation in the world's largest retrospective cohort study.

Br J Haematol 2019 Mar 28. Epub 2019 Mar 28.

Division of Haematology, Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan.

Reactivation of hepatitis B virus (HBV) by reverse seroconversion (HBV-RS) after allogeneic haematopoietic stem cell transplantation (allo-HSCT) can occur in patients with resolved HBV infection (rHBV, defined as negative HBV surface antigen [HBsAg] and positive HBV core antibody), and may cause fatal hepatitis. To explore the risk factors, we retrospectively identified 817 consecutive patients who underwent allo-HSCT from 2005 to 2016 in this largest single centre cohort from National Taiwan Univerisity Hospital. Transplants using donors or recipients positive for HBsAg or HBV DNA were excluded, leaving 445 rHBV patients for analysis. Read More

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http://dx.doi.org/10.1111/bjh.15884DOI Listing
March 2019
5 Reads

Risk of hospitalised bleeding in comparisons of oral anticoagulant options for the primary treatment of venous thromboembolism.

Br J Haematol 2019 Mar 28. Epub 2019 Mar 28.

Department of Epidemiology, Rollins School of Public Health, Emory University, Atlanta, GA, USA.

Understanding of the comparative bleeding risks of oral anticoagulant (OAC) therapies for the primary treatment of venous thromboembolism (VTE) is limited. Therefore, among anticoagulant-naïve VTE patients, we conducted comparisons of apixaban, rivaroxaban and warfarin on the rate of hospitalised bleeding within 180 days of OAC initation. MarketScan databases for the time-period from 2011 to 2016 were used and, for each OAC comparison, new users were matched with up to five initiators of a different OAC. Read More

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http://dx.doi.org/10.1111/bjh.15857DOI Listing
March 2019
1 Read

A phase III randomized, multicentre, double blind, active controlled trial to compare the efficacy and safety of two different anagrelide formulations in patients with essential thrombocythaemia - the TEAM-ET 2·0 trial.

Br J Haematol 2019 Mar 28. Epub 2019 Mar 28.

AOP Orphan Pharmaceuticals AG, Vienna, Austria.

Anagrelide is an established treatment option for essential thrombocythaemia (ET). A prolonged release formulation was developed with the aim of reducing dosing frequency and improving tolerability, without diminishing efficacy. This multicentre, randomized, double blind, active-controlled, non-inferiority trial investigated the efficacy, safety and tolerability of anagrelide prolonged release (A-PR) over a reference product in high-risk ET patients, either anagrelide-naïve or -experienced. Read More

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http://dx.doi.org/10.1111/bjh.15824DOI Listing

Lenalidomide plus rituximab (R ) in previously untreated marginal zone lymphoma: subgroup analysis and long-term follow-up of an open-label phase 2 trial.

Br J Haematol 2019 Mar 28. Epub 2019 Mar 28.

Department of Lymphoma/Myeloma, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.

Lack of consensus for first-line marginal zone lymphoma (MZL) treatment and toxicities associated with currently available systemic therapies have inspired evaluation of immunotherapeutic agents yielding robust outcomes with improved tolerability. We previously reported durable efficacy with first-line lenalidomide and rituximab (R ) in follicular lymphoma, MZL and small lymphocytic lymphoma with a subsequent long-term follow-up shown here in MZL patients. This phase 2 investigator-initiated study included previously untreated, stage III/IV MZL patients treated with lenalidomide 20 mg/day on days 1-21 and rituximab 375 mg/m on day 1 of each 28-day cycle, continuing in responders for ≥6-12 cycles. Read More

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http://dx.doi.org/10.1111/bjh.15843DOI Listing
March 2019
1 Read

Advances in understanding the molecular mechanisms that maintain normal haemostasis.

Br J Haematol 2019 Mar 28. Epub 2019 Mar 28.

Haemostasis Research Group, Department of Molecular and Cellular Therapeutics, Irish Centre for Vascular Biology, Royal College of Surgeons in Ireland, Dublin, Ireland.

Blood clot formation to stem bleeding from an injured blood vessel arises from a complex series of cellular and biochemical events, which, when dysregulated, predispose to an increased risk of thrombosis or bleeding. Similarly, haemostatic regulation of clot growth and size is exquisitely controlled by a series of anticoagulant 'checkpoints', that exert their inhibitory activity at distinct stages in the steps leading to clot formation. Although the major plasma protein constituents required for haemostasis have now been largely elucidated and the molecular events that lead to clot formation are well understood, defining a fuller appreciation of the importance, location and regulation of each haemostatic process remains a fertile area of ongoing research. Read More

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http://dx.doi.org/10.1111/bjh.15872DOI Listing

Comparison of Rituximab originator (MabThera) to biosimilar (Truxima) in patients with immune-mediated thrombotic thrombocytopenic purpura.

Br J Haematol 2019 Mar 28. Epub 2019 Mar 28.

Department of Haematology, UCLH NHS Foundation Trust, London, UK.

Immune thrombotic thrombocytopenic purpura (iTTP) is an acute, multisystem thrombotic microangiopathy mediated by ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) autoantibodies. Immunosuppression with anti-CD20 therapy is the mainstay of treatment. MabThera's patent has now expired and biosimilars have been approved. Read More

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http://dx.doi.org/10.1111/bjh.15874DOI Listing
March 2019
2 Reads

Real world experience at a single centre using low dose direct oral anticoagulants after unprovoked venous thromboembolism.

Br J Haematol 2019 Mar 28. Epub 2019 Mar 28.

Pulmonary Vascular Diseases Unit, Royal Papworth Hospital, Cambridgeshire, UK.

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http://dx.doi.org/10.1111/bjh.15880DOI Listing
March 2019
2 Reads

Autologous adipose-derived stem cell for painful leg ulcers in patients with sickle cell disease. A preliminary study.

Br J Haematol 2019 Mar 28. Epub 2019 Mar 28.

Division of Plastic Surgery, Department of Surgery and Anatomy, Hemocentro and Ribeirão Preto Medical School of University of São Paulo, Ribeirão Preto, São Paulo, Brazil.

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http://dx.doi.org/10.1111/bjh.15891DOI Listing
March 2019
1 Read

G-CSF administration prior to donor lymphocyte apheresis promotes anti-leukaemic effects in allogeneic HCT patients.

Br J Haematol 2019 Mar 27. Epub 2019 Mar 27.

Blood and Marrow Transplantation, Department of Medicine II, University Hospital Tuebingen, Tuebingen, Germany.

Donor lymphocyte infusion (DLI) is an effective method to establish full donor chimerism or to prevent and treat relapse after allogeneic haematopoietic cell transplantation (allo-HCT). Usually, DLIs are collected from naïve donors as steady-state lymphocytes. When donor lymphocytes are collected during stem cell apheresis, donors are pre-treated with granulocyte colony-stimulating factor (G-CSF). Read More

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http://dx.doi.org/10.1111/bjh.15881DOI Listing

Atypical haemolytic uraemic syndrome in the eculizumab era: presentation, response to treatment and evaluation of an eculizumab withdrawal strategy.

Br J Haematol 2019 Mar 27. Epub 2019 Mar 27.

Department of Haematology, UCLH, Cardiometabolic programme- NIHR UCLH/UCL BRC, London, UK.

The complement inhibitor, eculizumab, has revolutionised the management of atypical haemolytic uraemic syndrome (aHUS), although the optimum treatment duration is debated. Twenty-two cases of acute aHUS managed with eculizumab were retrospectively reviewed, including outcomes after eculizumab withdrawal. Although 41% had an associated complement genetic abnormality, mutation status did not affect severity of clinical presentation. Read More

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http://dx.doi.org/10.1111/bjh.15899DOI Listing
March 2019
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The poor prognosis of low hypodiploidy in adults with B-cell precursor acute lymphoblastic leukaemia is restricted to older adults and elderly patients.

Br J Haematol 2019 Mar 27. Epub 2019 Mar 27.

Institut de Recerca contra la Leucemia Josep Carreras, Hospital Germans Trias i Pujol, Universitat Autònoma de Barcelona, Badalona, Spain.

The prognostic significance of low-hypodiploidy has not been extensively evaluated in minimal residual disease (MRD)-oriented protocols for adult acute lymphoblastic leukaemia (ALL). We analysed the outcome of hypodiploid adult ALL patients treated within Programa Español de Tratamientos en Hematología (PETHEMA) protocols. The 5-year cumulative incidence of relapse (CIR) of low-hypodiploid B-cell precursor (BCP)-ALL was significantly higher than that of high-hypodiploids (52% vs. Read More

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http://dx.doi.org/10.1111/bjh.15887DOI Listing
March 2019
1 Read

Stigmata of amyloidosis; external manifestations of internal disease.

Br J Haematol 2019 Mar 27. Epub 2019 Mar 27.

Tufts Medical Center and Tufts University School of Medicine, Boston, MA, USA.

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http://dx.doi.org/10.1111/bjh.15885DOI Listing

S100A16 suppresses the growth and survival of leukaemia cells and correlates with relapse and relapse free survival in adults with Philadelphia chromosome-negative B-cell acute lymphoblastic leukaemia.

Br J Haematol 2019 Mar 27. Epub 2019 Mar 27.

Peking University People's Hospital, Peking University Institute of Haematology, Beijing, China.

Refinement of risk stratification in Philadelphia chromosome (Ph)-negative B-cell acute lymphoblastic leukaemia (ALL) might aid the identification of patients who are likely to relapse. Abnormal S100 calcium binding protein A16 (S100A16) has been implicated in various cancers, but its function remains unclear. We found S100A16 transcript levels were higher in 130 adults with newly-diagnosed Ph-negative B-cell ALL compared with 33 healthy controls. Read More

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http://dx.doi.org/10.1111/bjh.15878DOI Listing
March 2019
2 Reads
4.711 Impact Factor