Semin Thromb Hemost 2012; 38(05): 469-482
DOI: 10.1055/s-0032-1306431
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Autoimmune Thrombotic Microangiopathy: Advances in Pathogenesis, Diagnosis, and Management

Han-Mou Tsai
1   Division of Hematology-Oncology, Pennsylvania State University, Milton S. Hershey Medical Center, Hershey, Pennsylvania
› Author Affiliations
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Publication History

Publication Date:
03 April 2012 (online)

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Abstract

Thrombotic microangiopathy, or the syndrome of thrombocytopenia and hemolysis with schistocytes on blood smears, has been a subject of uncertainty and intense controversy. The pathogenesis of thrombotic microangiopathy was unknown and no classification of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome was satisfactory. In recent years, a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS-13) deficiency and defective complement regulation have been identified as the two major causes of noninfectious thrombotic microangiopathy. It is now possible to classify thrombotic microangiopathy pathogenetically rather than clinically, and a distinction between diseases and clinical syndromes is emerging. This pathogenesis-based disease classification requires new diagnostic approaches and provides a framework for rational therapeutic designs. This review discusses the new concepts in the pathogenesis, diagnosis, and management of thrombotic microangiopathy, with particular emphasis on the autoimmune causes of ADAMTS-13 deficiency and defective complement regulation.