title: von Willebrand's variant (type II Buffalo). Thrombocytopenia after desmopressin but absence of in vitro hypersensitivity to ristocetin.
abstract: Von Willebrand's disease is categorized into types and subtypes based on multimeric analysis of plasma von Willebrand's factor. Such categorization is of value because both the mode of inheritance and the choice of therapeutic material differ between subtypes. The Type IIB variant is characterized by hypersensitivity in vitro to ristocetin and thrombocytopenia after administration of desmopressin (DDAVP). Hypersensitivity to ristocetin has also been described in Type I variants but without thrombocytopenia after DDAVP. This report describes a new Type II variant characterized by the converse situation, absence of hypersensitivity to ristocetin in vitro but transient thrombocytopenia after intravenous administration of DDAVP.
authors.affiliation: Department of Hematology, Buffalo General Hospital 14215.
authors.forename: J D
authors.initials: JD
authors.lastname: Sweeney
authors.forename: L A
authors.initials: LA
authors.lastname: Hoernig
authors.forename: A N
authors.initials: AN
authors.lastname: Behrens
authors.forename: E
authors.initials: E
authors.lastname: Novak
authors.forename: R T
authors.initials: RT
authors.lastname: Swank
mesh_terms: D003894:Deamino Arginine Vasopressin / Q000008:administration & dosage / Q000009:adverse effects*; D004342:Drug Hypersensitivity / Q000097:blood / Q000209:etiology*; D005260:Female; D006801:Humans; D007262:Infusions, Intravenous; D008297:Male; D010375:Pedigree; D010974:Platelet Aggregation / Q000187:drug effects; D010976:Platelet Count; D012310:Ristocetin / Q000009:adverse effects*; D013921:Thrombocytopenia / Q000139:chemically induced*; D014842:von Willebrand Diseases / Q000097:blood* / Q000145:classification / Q000235:genetics; D014841:von Willebrand Factor / Q000032:analysis / Q000235:genetics
chemical_list: D014841:von Willebrand Factor; D012310:Ristocetin; D003894:Deamino Arginine Vasopressin