Is there a shared pathophysiology for thrombotic thrombocytopenic purpura and hemolytic-uremic syndrome?
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.
Department of Pediatrics, University of Michigan, Life Sciences Institute, Ann Arbor, Michigan, USA.
Karl
K
Desch
David
D
Motto
D051722:ADAM Proteins / Q000378:metabolism; D000071120:ADAMTS13 Protein; D004730:Endothelium, Vascular / Q000503:physiopathology; D006463:Hemolytic-Uremic Syndrome / Q000209:etiology / Q000503:physiopathology*; D006801:Humans; D007668:Kidney / Q000098:blood supply; D011697:Purpura, Thrombotic Thrombocytopenic / Q000209:etiology / Q000503:physiopathology*; D022621:Shiga Toxin / Q000378:metabolism; D014841:von Willebrand Factor / Q000378:metabolism
D014841:von Willebrand Factor; D022621:Shiga Toxin; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human