title: Autoimmune thrombotic microangiopathy: advances in pathogenesis, diagnosis, and management.
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.
authors.affiliation: Pennsylvania State University, Milton S. Hershey Medical Center, Hershey, PA, USA. htsai@hmc.psu.edu
authors.forename: Han-Mou
authors.initials: HM
authors.lastname: Tsai
mesh_terms: D051722:ADAM Proteins / Q000097:blood / Q000172:deficiency; D000071120:ADAMTS13 Protein; D001327:Autoimmune Diseases / Q000097:blood / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D019468:Disease Management; D006801:Humans; D057049:Thrombotic Microangiopathies / Q000097:blood / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy*
chemical_list: D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human