Factor VIII-related activities after 1-deamino-8-D-arginine vasopressin in multicentric reticulohistiocytosis

Abstract
Summary A woman with multicentric reticulohistiocytosis, having symptoms including bleeding and a borderline level of factor VIII, was given an i.v. infusion of DDAVP as a pharmacological test to evaluate the release of factor VIII-related properties from storage sites. The results show a reduced response to DDAVP in the patient in comparison with a normal control subject. This could be due either to a defect in the release of the factor VIII complex after DDAVP or to a reduced synthesis and/or storage capacity by the storage cells.