title: The thrombotic thrombocytopenic purpura and hemolytic uremic syndromes: overview of pathogenesis (Experience of The Oklahoma TTP-HUS Registry, 1989-2007).
abstract: The Oklahoma TTP (thrombotic thrombocytopenic purpura)-HUS (hemolytic uremic syndrome) Registry, an inception cohort of 382 consecutive patients with TTP-HUS, provides a complete community perspective of these syndromes. TTP is the diagnostic term used for all adults, with or without neurologic or renal abnormalities; it is typically an acquired disorder; it may rarely result from congenital ADAMTS13 deficiency. HUS is the term used for children who have renal failure, most often caused by Escherichia coli O157:H7 infection; it may rarely result from congenital abnormalities of complement regulation. Clinical categories related to associated conditions and potential etiologies provide a structure for describing pathogenesis of the acquired syndromes. (1) Following allogeneic hematopoietic stem cell transplantation; a disorder primarily affecting kidneys described as transplantation-associated thrombotic microangiopathy. (2) Pregnancy-associated; pregnancy is a prominent risk factor for the development of TTP. (3) Drug-associated; acute, immune-mediated systemic syndromes and also dose-dependent renal toxicity. (4) Bloody diarrhea prodrome, suggesting an enteric infectious etiology. (5) Presence of an additional autoimmune disorder. (6) Idiopathic. A severe deficiency of ADAMTS13 activity contributes to the pathogenesis of many idiopathic patients and also some patients who present during pregnancy, with bloody diarrhea, or who have additional autoimmune disorders.
authors.affiliation: Department of Medicine, The University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma 73126-0901, USA. james-george@ouhsc.edu
authors.forename: James N
authors.initials: JN
authors.lastname: George
mesh_terms: D051722:ADAM Proteins / Q000172:deficiency; D000071120:ADAMTS13 Protein; D001327:Autoimmune Diseases / Q000150:complications; D003967:Diarrhea / Q000382:microbiology; D064420:Drug-Related Side Effects and Adverse Reactions; D019453:Escherichia coli O157 / Q000472:pathogenicity; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects; D006463:Hemolytic-Uremic Syndrome / Q000145:classification / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology*; D006801:Humans; D008297:Male; D009825:Oklahoma / Q000453:epidemiology; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000209:etiology; D011697:Purpura, Thrombotic Thrombocytopenic / Q000145:classification / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology*; D012042:Registries; D012307:Risk Factors; D009626:Terminology as Topic
chemical_list: D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human