Publications by authors named "B Joly"

Thrombotic Thrombocytopenic Purpura (TTP) is a rare disease characterized by a severe deficiency of ADAMTS13, the specific protease that cleaves von Willebrand factor. The congenital form of TTP (cTTP) results from pathogenic variants of the ADAMTS13 gene. cTTP has two peaks of incidence: one in childhood and the other in adulthood, mainly in an obstetric context.

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Introduction: Multiple myeloma patients aged 80 years and older are a population more prone to comorbidities and frailty. We aim to describe the real-life management and outcomes of this population. EMMY is a descriptive large-scale study.

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Article Synopsis
  • * This study explores the clinical presentation and outcomes of patients with uTTP, highlighting similarities to immune TTP (iTTP).
  • * Key features like young age, brain involvement, and severe low platelet counts, especially in those with a history of autoimmune disease or pregnancy, should raise suspicion for iTTP diagnosis.
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