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.
Subject(s)
Deamino Arginine Vasopressin/adverse effects , Drug Hypersensitivity/etiology , Ristocetin/adverse effects , Thrombocytopenia/chemically induced , von Willebrand Diseases/blood , Deamino Arginine Vasopressin/administration & dosage , Drug Hypersensitivity/blood , Female , Humans , Infusions, Intravenous , Male , Pedigree , Platelet Aggregation/drug effects , Platelet Count , von Willebrand Diseases/classification , von Willebrand Diseases/genetics , von Willebrand Factor/analysis , von Willebrand Factor/geneticsABSTRACT
A 73-year-old woman developed an acquired factor VIII inhibitor in association with squamous cell carcinoma of the epiglottis. The inhibitor was an IgG antibody that reacted with factor VIII in vitro and in vivo. Intravenous gamma-globulin therapy was successful in reducing the inhibitor so that curative surgery could be undertaken. With surgical resection of the tumor the inhibitor did not recur. The relevance of this type of coagulation disorder to the surgical management of the patient's head and neck cancer is discussed.
