title: Effect of splenectomy on von Willebrand factor multimeric structure in thrombotic thrombocytopenic purpura refractory to plasma exchange.
abstract: Treatment-related changes in plasma von Willebrand factor (vWf) multimers were studied in two patients with thrombotic thrombocytopenic purpura (TTP) undergoing splenectomy because of refractoriness to plasma exchange. In both cases, a decrease in the largest normal vWf multimers was found at presentation. In one case, splenectomy resulted in a long-term remission and was followed by a normal multimeric pattern. In the other case, splenectomy failed to produce a remission and was followed by the appearance of ultralarge multimers, which disappeared--resulting in a normal pattern--when a sustained remission was achieved after vincristine infusion and further plasma exchanges. These results suggest that splenectomy per se has no consistent effect on the vWf multimeric structure in TTP patients, and provide further evidence on the good prognostic value of the achievement of a normal multimeric pattern in these patients.
authors.affiliation: Department of Hemotherapy-Hemostasis, Hospital Clínico, Barcelona, Spain.
authors.forename: A
authors.initials: A
authors.lastname: Pereira
authors.forename: J
authors.initials: J
authors.lastname: Monteagudo
authors.forename: A
authors.initials: A
authors.lastname: Bono
authors.forename: A
authors.initials: A
authors.lastname: Lopez-Guillermo
authors.forename: A
authors.initials: A
authors.lastname: Ordinas
mesh_terms: D000293:Adolescent; D004591:Electrophoresis, Polyacrylamide Gel; D005260:Female; D006801:Humans; D046911:Macromolecular Substances; D008875:Middle Aged; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000097:blood / Q000601:surgery / Q000628:therapy*; D013156:Splenectomy; D014841:von Willebrand Factor / Q000737:chemistry*
chemical_list: D046911:Macromolecular Substances; D014841:von Willebrand Factor