Publications by authors named "Pierre Edouard Debureaux"

Background: Venetoclax is the first representative of a new class of targeted therapy, that inhibits selectively B-cell lymphoma-2 (BCL-2), an anti-apoptotic protein, frequently overexpressed in hematological malignancies. Venetoclax was approved by the Food and Drug Administration for chronic lymphocytic leukemia and for acute myeloid leukemia in 2016 and 2021, respectively. Because of its promising role in many hematological malignancies, several clinical trials are in progress and other extensions of indication are expected.

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Inflammatory form of Waldenström macroglobulinemia (iWM) predicts outcomes after immuno-chemotherapy and Bruton tyrosine kinase inhibitors, but its origin is unknown. Here, we unravel increased clonal hematopoiesis in patients with iWM (61% vs 23% in noninflammatory WM), suggesting a contribution of environmental cells to iWM.

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Seric hyperviscosity syndrome is a medical emergency linked to hyperproteinemia. The clinical diagnosis hinges on a triad of symptoms: mucosal hemorrhages, visual disturbances, and neurological disorders, observed in the most severe cases. Diagnosis is swiftly confirmed through an urgent fundoscopic examination.

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A second autologous stem-cell transplantation (ASCT2) is considered for relapsed multiple myeloma (RMM) patients showing prolonged response after a first ASCT. However, given breakthrough treatments like anti-CD38 and immunotherapy, its role remains debated. We conducted a real-life study in 10 French centers (1996-2017) involving 267 RMM patients receiving ASCT2.

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Article Synopsis
  • The role and timing of allogeneic hematopoietic stem cell transplantation (HSCT) in treating acute myelogenous leukemia (AML) have been debated for decades, with reliance on the European LeukemiaNet classification for treatment strategies.
  • A study found that HSCT significantly improved overall survival for intermediate- and poor-risk AML patients, particularly younger patients, while showing low cumulative incidence rates for older groups due to factors like comorbidities and eligibility.
  • With increasing access to various donor types, including haploidentical ones, the role of HSCT in AML treatment may evolve, potentially increasing transplant numbers in adult patients.
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  • Late relapse (LR) after a special blood stem cell treatment for leukemia happens in about 4.5% of patients and is studied to find out how well they do after this happens.
  • A study looked at 7,582 patients who had the treatment, and 319 of them had late relapses, mostly after about 38 months.
  • The chances of living longer after a late relapse were about 19.9 months, with many patients getting better after a second treatment.
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  • * Despite the promising survival rates, the treatment had a high toxicity level, leading to an 11% mortality rate from toxicity and 21% treatment-related mortality overall, particularly affecting patients aged 60 and older.
  • * The findings suggest that future research should focus on identifying patients who will benefit the most from this treatment and finding ways to decrease the toxicity of the conditioning regimen.
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Patients with paroxysmal nocturnal hemoglobinuria (PNH) are susceptible to complement-mediated intravascular hemolysis and thrombosis. Factor H (FH) is the main regulator of the complement alternative pathway, which protects cells from unwanted complement-mediated damage. Although FH is not a glycosylphosphatidylinositol-linked molecule, it may play a role in PNH.

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Hyperviscosity syndrome (HVS) is a rare complication of newly diagnosed multiple myeloma (NDMM) related to high tumour burden. Studies about the prognosis of HVS in modern-era therapy for NDMM are missing. We investigated a retrospective cohort study of NDMM with HVS between 2011-2021.

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Waldenström's macroglobulinaemia (WM) is a B-cell neoplasm resulting from bone marrow lymphoplasmacytic infiltration and monoclonal IgM secretion. Some patients present concomitant inflammatory syndrome attributed to the disease activity; we named this syndrome inflammatory WM (IWM). We retrospectively analysed all WM patients seen in a single tertiary referral centre from January 2007 to May 2021, and after excluding aetiologies for the inflammatory syndrome using a pertinent blood workup, including C-reactive protein (CRP), and imaging, we identified 67 (28%) IWM, 166 (68%) non-IWM, and nine (4%) WM with inflammatory syndrome of unknown origin.

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  • Sinusoidal obstruction syndrome (SOS) is a dangerous liver problem that can happen after high-dose chemotherapy, and the only approved treatment for it is defibrotide.
  • In a study of 71 patients in tough medical situations due to SOS, many needed help for their breathing and kidneys, and 54% sadly did not survive the hospital stay.
  • Older patients and those needing special machines to help with breathing or kidney function had a higher risk of dying, but using defibrotide for prevention seemed to help some patients do better.
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  • * A study involving 80 MPN-SVT patients over an 11-year median follow-up found that 13% experienced severe hematologic outcomes, linked to higher JAK2 mutation levels and other genetic mutations.
  • * High-risk patients, making up 29% of the cohort and showing significant molecular risk factors, had poorer survival rates, suggesting the need for targeted therapy to prevent disease progression.
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Significant morbidity and mortality have been associated with liver complications after allogeneic hematopoietic stem cell transplantation (allo-HSCT). Causes and consequences of these hepato-biliary complications are various and might be life-threatening. A high misdiagnosis rate has been reported because of a weak correlation between clinical, laboratory and imaging data.

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Optimization of the salvage regimen is required to improve prognosis in primary refractory or relapsed acute myeloid leukemia (AML). In fit patients, a bridge to allogeneic transplant is the primary purpose of salvage. We tested the combination of fractionated gemtuzumab ozogamicin with cytarabine and mitoxantrone (MYLODAM schema) with primary endpoint of efficacy and safety.

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