1,726 results match your criteria: "Department of Clinical Haematology[Affiliation]"

An international survey to better understand the current incidence, severity, and management of VOD/SOS.

Bone Marrow Transplant

January 2025

Sorbonne Université, Centre de Recherche Saint-Antoine INSERM UMRs938, Service d'Hématologie Clinique et de Thérapie Cellulaire, Hôpital Saint Antoine, AP-HP, Paris, France.

Article Synopsis
  • * A significant number of respondents (67.0%) felt that early diagnosis was challenging, but many (75.8%) found the new 2023 EBMT diagnostic criteria useful and easy to apply.
  • * Key risk factors for VOD/SOS included second allo-HCT, pre-existing liver disease, and prior use of antibody-drug conjugates, with varied preferences on when to start treatment.
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Should we screen for plasma cell dyscrasias in people with low bone density?

Lancet Haematol

October 2024

Division of Hematology and Hematological Malignancies, Huntsman Cancer Institute, University of Utah, Salt Lake City, UT 84093, USA. Electronic address:

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Position statement on the management of pregnancy in sickle cell disease.

Aust N Z J Obstet Gynaecol

September 2024

Schools of Medicine and Women's and Children's Health, Prince of Wales Hospital and Royal Hospital for Women Clinical School, University of NSW, Sydney, New South Wales, Australia.

Sickle cell disease (SCD) is a hereditary haemoglobinopathy which causes multi-organ dysfunction. Pregnancies in SCD are high risk with significant maternal and fetal morbidity and mortality, including vaso-occlusive crises, thrombosis, anaemia, placental insufficiency, fetal growth restriction, preterm birth and medication effects. High level evidence on this topic is lacking.

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Background: Invasive fungal disease (IFD) is a significant complication for children receiving treatment for leukaemia, contributing to morbidity and mortality. Recent regional paediatric epidemiological IFD data are lacking. Additionally uncertainty remains regarding the optimal prophylactic approach in this context.

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Introduction: When Coronavirus Disease-19 (COVID-19) struck the world in December 2019, initiatives started to investigate the efficacy of convalescent plasma, a readily available source of passive antibodies, collected from recovered patients as a therapeutic option. This was based on historical observational data from previous virus outbreaks.

Methods: A scoping review was conducted on the efficacy and safety of convalescent plasma and hyperimmune immunoglobulins for COVID-19 treatment.

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Introduction: Immune effector cell-associated neurotoxicity syndrome (ICANS) is a common side-effect of chimeric antigen receptor T-cell (CAR-T) therapy, with symptoms ranging from mild to occasionally life-threatening. The neurological, cognitive, psychiatric and psychosocial sequelae of ICANS are diverse and not well defined, posing a challenge for diagnosis and management. The recovery trajectory of the syndrome is uncertain.

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Objectives: Haematopoietic stem cell transplant (HCT) is a cellular therapy that, whilst curative for a child's underlying disease, carries significant risk of mortality, including because of pulmonary complications. The aims of this study were to describe the burden of pulmonary complications post-HCT in a cohort of Australian children and identify risk factors for the development of these complications.

Methods: Patients were identified from the HCT databases at two paediatric transplant centres in Australia.

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Acute lymphoblastic leukaemia (ALL) in 20%-30% of adult patients contains the Philadelphia (Ph+) chromosome. Historically, Ph+ ALL denoted a markedly inferior outcome and long-term survival in the absence of an allograft was uncommon. However, the advent of targeted therapy directed against the BCR::ABL1 fusion protein with various tyrosine kinase inhibitors (TKIs) has markedly improved the prognosis, resulting in a number of treatment controversies in allograft-eligible patients.

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Gilteritinib is the current standard of care for relapsed or refractory fms related receptor tyrosine kinase 3 (FLT3)-mutated acute myeloid leukemia in many countries, however outcomes for patients relapsing after contemporary first-line therapies (intensive chemotherapy with midostaurin, or nonintensive chemotherapy with venetoclax) are uncertain. Moreover, reported data on toxicity and health care resource use is limited. Here, we describe a large real-world cohort of 152 patients receiving single-agent gilteritinib in 38 UK hospitals.

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Objective: To determine the effectiveness of cyclosporin A (CSA) monotherapy in treating patients with non-severe aplastic anaemia (NSAA).

Study Design: A cross-sectional observational study. Place and Duration of the Study: Department of Clinical Haematology, Armed Forces Bone Marrow Transplant Center, Rawalpindi, Pakistan, from January 2022 till December 2023.

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The changing face of cerebral venous sinus thrombosis-emerging new causes and treatments.

J Thromb Haemost

December 2024

Thrombosis and Haemophilia Centre, Guy's & St Thomas' NHS Foundation Trust, London, United Kingdom. Electronic address: https://twitter.com/bhwords.

Article Synopsis
  • * Risk factors for CVST include hormonal factors like birth control and pregnancy, along with new ones such as obesity, COVID-19, and vaccine-related complications.
  • * Treatment mainly involves anticoagulation and addressing underlying causes, with severe cases potentially requiring invasive procedures like thrombolysis or craniectomy.
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Background: Many patients with hematological malignancy develop fever after chemotherapy/conditioning but before chemotherapy-induced neutropenia (preneutropenic fever [PNF]). The proportion of PNF with an infectious etiology is not well established.

Methods: We conducted a single-center, prospective observational substudy of PNF (neutrophils >0.

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Article Synopsis
  • The EHA-ESMO Clinical Practice Guideline offers important recommendations for the diagnosis, staging, treatment, and follow-up of HIV-associated lymphomas.
  • It includes insights from a diverse team of experts in oncology from various European institutions.
  • The recommendations are grounded in scientific research and the collective expertise of the authors.
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Article Synopsis
  • - Mastocytosis is a disorder characterized by an abnormal increase of mast cells in one or more organs, with varying symptoms and outcomes; it's classified into cutaneous, systemic, and MC sarcoma types by the WHO.
  • - The disease often involves a specific mutation (D816V) in most systemic mastocytosis cases, affecting life expectancy significantly based on the type, with those having nonadvanced forms generally living near-normal lifespans, while advanced forms have limited life expectancies.
  • - Recently, a group of experts proposed updated diagnostic criteria and classifications for mastocytosis, aiming to unify different classifications from previous organizations to improve research and clinical comparisons.
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Inherited thrombocytopenias (ITs) encompass a group of rare disorders characterized by diminished platelet count. Recent advancements have unveiled various forms of IT, with inherited thrombocytopenia 2 (THC2) emerging as a prevalent subtype associated with germline variants in the critical 5' untranslated region of the gene. This region is crucial in regulating the gene expression of , particularly in megakaryocytes.

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Patients with Waldenström macroglobulinemia have impaired platelet and coagulation function.

Blood Adv

November 2024

Division of Genome Science and Cancer, John Curtin School of Medical Research, The Australian National University, Canberra, ACT, Australia.

Article Synopsis
  • Patients with Waldenström macroglobulinemia (WM) experience symptoms like cytopenias, fatigue, and bleeding, often exacerbated by therapeutics like Bruton's tyrosine kinase inhibitors (BTKis).
  • A study analyzed hemostatic dysfunction in WM patients, comparing samples from untreated patients, those on BTKis, and healthy donors, using various blood tests to assess platelet function and clotting potential.
  • Results showed that WM patients had decreased platelet reticulation, slower thrombin generation, and impaired hemostatic function due to high levels of IgM, indicating serious disturbances in blood coagulation despite receiving treatment.
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Article Synopsis
  • Paroxysmal nocturnal haemoglobinuria (PNH) is a rare blood disorder causing serious issues due to chronic hemolysis and can significantly affect patients' quality of life.
  • A post hoc analysis evaluated the effectiveness of pegcetacoplan, a targeted complement C3 inhibitor, in PNH patients with poor bone marrow function from the PEGASUS and PRINCE studies.
  • Results showed that while normalisation of certain health parameters was challenging, a significant percentage of patients experienced clinically meaningful improvements in hemoglobin, LDH levels, and fatigue after treatment with pegcetacoplan.
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Thrombocytopenic patients have an increased risk of bleeding when undergoing invasive procedures. In a multicentre, phase II, blinded, randomised, controlled feasibility trial, critically ill patients with platelet count 100 × 10/L or less were randomised 1:1 to intravenous desmopressin (0.3 µg/kg) or placebo before an invasive procedure.

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Article Synopsis
  • - Pain is a common issue in dermatology that can impact daily activities and overall quality of life, varying in intensity from mild to severe across different skin conditions.
  • - Serious skin diseases like pemphigus vulgaris and Stevens-Johnson syndrome require a team approach to pain management, often involving pain specialists and addressing both nociceptive and neuropathic pain types.
  • - Effective pain management strategies include medications ranging from non-opioids to opioids, guided by the WHO analgesic ladder, but there is a lack of research and awareness surrounding this topic in dermatology.
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Objective: To characterize surface-bound proteins and to measure the thickness of fibrin fibers bound to extracorporeal membrane oxygenation (ECMO) circuits used in children.

Design: Single-center observational prospective study, April to November 2021.

Setting: PICU, Royal Children's Hospital, Melbourne, Australia.

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Prior studies have suggested that immune thrombotic thrombocytopenic purpura (iTTP) may display seasonal variation; however, methodologic limitations and sample sizes have diminished the ability to perform a rigorous assessment. This 5-year retrospective study assessed the epidemiology of iTTP and determined whether it displays a seasonal pattern. Patients with both initial and relapsed iTTP (defined as a disintegrin and metalloprotease with thrombospondin type motifs 13 activity <10%) from 24 tertiary centers in Australia, Canada, France, Greece, Italy, Spain, and the US were included.

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