title: Relapse of thrombotic thrombocytopenic purpura associated with decreased VWF cleaving activity.
abstract: Thrombotic thrombocytopenic purpura (TTP) is an uncommon syndrome characterized by reversible, systemic aggregation of platelets in the microcirculation and disseminated microvascular thrombosis. Surgery may precipitate TTP and has been associated with relapse in some patients. However, relapse of this life-threatening disorder is unpredictable. We report a patient with an antecedent history of TTP who experienced a relapse after elective cardiac surgery. In this case, decreased von Willebrand factor (vWF)-cleaving metalloproteinase activity and an inhibitor of this endogenous enzyme were demonstrated preoperatively. These findings suggest that decreased vWF-cleaving metalloproteinase activity and/or the presence of its inhibitor may predict an increased risk for surgical-associated relapse of TTP.
authors.affiliation: manstadt@mail.mcg.edu
authors.forename: Mark P
authors.initials: MP
authors.lastname: Anstadt
authors.forename: John M
authors.initials: JM
authors.lastname: Carwile
authors.forename: Carrie K
authors.initials: CK
authors.lastname: Guill
authors.forename: Lori D
authors.initials: LD
authors.lastname: Conklin
authors.forename: Ernesto R
authors.initials: ER
authors.lastname: Soltero
authors.forename: Anthony
authors.initials: A
authors.lastname: Lucci
authors.forename: Michael H
authors.initials: MH
authors.lastname: Kroll
mesh_terms: D000328:Adult; D001026:Coronary Artery Bypass / Q000009:adverse effects*; D006801:Humans; D008297:Male; D008666:Metalloendopeptidases / Q000097:blood; D010974:Platelet Aggregation; D011697:Purpura, Thrombotic Thrombocytopenic / Q000097:blood* / Q000209:etiology* / Q000628:therapy; D012008:Recurrence; D012307:Risk Factors; D014841:von Willebrand Factor / Q000378:metabolism*
chemical_list: D014841:von Willebrand Factor; D008666:Metalloendopeptidases