Anti-ADAMTS13 Autoantibodies: From Pathophysiology to Prognostic Impact-A Review for Clinicians

Cristina Dainese; Federica Valeri; Benedetto Bruno; Alessandra Borchiellini

doi:10.3390/jcm12175630

Anti-ADAMTS13 Autoantibodies: From Pathophysiology to Prognostic Impact-A Review for Clinicians

J Clin Med. 2023 Aug 29;12(17):5630. doi: 10.3390/jcm12175630.

Authors

Cristina Dainese^{1

2}, Federica Valeri^{1

2}, Benedetto Bruno^{2

3}, Alessandra Borchiellini^{1

2}

Affiliations

¹ Regional Centre for Hemorrhagic and Thrombotic Diseases, AOU Città Della Salute e Della Scienza, 10126 Turin, Italy.
² Division of Hematology, AOU Città Della Salute e Della Scienza and University of Turin, 10124 Turin, Italy.
³ Department of Molecular Biotechnology and Health Sciences, University of Turin, 10124 Turin, Italy.

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 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.

Keywords: ADAMTS13 autoantibodies; ADAMTS13 inhibitors; Moskowitz syndrome; thrombotic thrombocytopenic purpura.

Publication types

Review

Grants and funding

This research received no external funding.