Overview

Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy caused by congenital or acquired severe deficiency of the von Willebrand factor cleaving protease, ADAMTS13. Acute TTP is a medical emergency requiring a rapid differential diagnosis and immediate treatment, which is often challenging due to the overlap between clinical and laboratory features and other thrombotic microangiopathies. Diagnostic and therapeutic advancements over the past decade, including the increased availability of ADAMTS13 testing and the development of caplacizumab, the first targeted therapy for acquired TTP, have significantly improved the management of acute TTP and reduced the burden of short-term outcomes such as mortality and exacerbation of acute disease. Concurrently, new clinical challenges have emerged, to mention but one, addressing the burden of long-term complications of TTP and cardiovascular comorbidities in TTP patients in remission.This Special Issue will cover new diagnostic and therapeutic aspects of TTP care, with the scope of advancing our knowledge and clinical intervention to address the current challenges of TTP management. Original, review, and guidance/guideline papers are welcome.

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9783725828593

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