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Keywords = Moskowitz syndrome

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10 pages, 458 KB  
Review
Anti-ADAMTS13 Autoantibodies: From Pathophysiology to Prognostic Impact—A Review for Clinicians
by Cristina Dainese, Federica Valeri, Benedetto Bruno and Alessandra Borchiellini
J. Clin. Med. 2023, 12(17), 5630; https://doi.org/10.3390/jcm12175630 - 29 Aug 2023
Cited by 5 | Viewed by 2922
Abstract
Thrombotic thrombocytopenic purpura (TTP) is a fatal disease in which platelet-rich microthrombi cause end-organ ischemia and damage. TTP is caused by markedly reduced ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) activity. ADAMTS13 autoantibodies (autoAbs) are the major [...] Read more.
Thrombotic thrombocytopenic purpura (TTP) is a fatal disease in which platelet-rich microthrombi cause end-organ ischemia and damage. TTP is caused by markedly reduced ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) activity. ADAMTS13 autoantibodies (autoAbs) are the major cause of immune TTP (iTTP), determining ADAMTS13 deficiency. The pathophysiology of such autoAbs as well as their prognostic role are continuous objects of scientific studies in iTTP fields. This review aims to provide clinicians with the basic information and updates on autoAbs’ structure and function, how they are typically detected in the laboratory and their prognostic implications. This information could be useful in clinical practice and contribute to future research implementations on this specific topic. Full article
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