title: Acquired von Willebrand's disease in association with essential thrombocythemia: regression following treatment.
abstract: Acquired von Willebrand's disease in a 40-year-old woman affected with essential thrombocythemia (ET) is reported. The profile of plasma von Willebrand factor (vWF) revealed decreased ristocetin cofactor activity and diminished large multimers of vWF in spite of a normal vWF antigen level. There was no evidence of circulating inhibitor against the factor VIII complex. The vWF abnormality improved by controlling the platelet count following treatment for ET with interferon-alpha 2b and ranimustine. The possible mechanism of the development of AvWD in ET is briefly discussed.
authors.affiliation: First Department of Internal Medicine, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Murakawa
authors.forename: T
authors.initials: T
authors.lastname: Okamura
authors.forename: K
authors.initials: K
authors.lastname: Tsutsumi
authors.forename: S
authors.initials: S
authors.lastname: Tanoguchi
authors.forename: T
authors.initials: T
authors.lastname: Kamura
authors.forename: T
authors.initials: T
authors.lastname: Shibuya
authors.forename: M
authors.initials: M
authors.lastname: Harada
authors.forename: Y
authors.initials: Y
authors.lastname: Niho
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D000077190:Interferon alpha-2; D016898:Interferon-alpha / Q000627:therapeutic use; D010974:Platelet Aggregation; D011994:Recombinant Proteins; D012310:Ristocetin / Q000097:blood; D013920:Thrombocythemia, Essential / Q000097:blood / Q000150:complications* / Q000628:therapy; D014842:von Willebrand Diseases / Q000097:blood / Q000209:etiology* / Q000628:therapy; D014841:von Willebrand Factor / Q000378:metabolism
chemical_list: D000077190:Interferon alpha-2; D016898:Interferon-alpha; D011994:Recombinant Proteins; D014841:von Willebrand Factor; D012310:Ristocetin