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Is There a Shared Pathophysiology for Thrombotic Thrombocytopenic Purpura and Hemolytic-Uremic Syndrome?

Karl Desch^* and David Motto

Department of Pediatrics, University of Michigan, Life Sciences Institute, Ann Arbor, Michigan; and Departments of Internal Medicine and Pediatrics, University of Iowa, Iowa City, Iowa

Correspondence: Dr. David Motto, Departments of Internal Medicine and Pediatrics, University of Iowa, 3269C Carver Biomedical Research Building, Iowa City, IA 52242. Phone: 319-353-5630; Fax: 319-353-8383; E-mail: david-motto{at}uiowa.edu

Thrombotic microangiopathy is characterized by microvascular thrombosis coupled with thrombocytopenia, hemolytic anemia, and red blood cell fragmentation. Familiar to nephrologists and hematologists alike, classically associated with thrombotic microangiopathy are the hemolytic-uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP), the histories and presentations of which are closely intertwined. Not surprising, these two disorders are considered by many to be manifestations of the same disease process, whereas others consider HUS and TTP to be distinct clinical and pathologic entities. Herein are reviewed HUS and TTP along with recent progress shedding new light on possible shared pathophysiologic mechanisms for these two intriguing disorders.

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Copyright © 2009 by the American Society of Nephrology. Online ISSN: 1533-3450 Print ISSN: 1046-6673