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We have studied a 17 month old boy with previously diagnosed vWD who presented with intracerebral hemorrhage. There was a history of excessive bleeding from circumcision, after corrective surgery for pyloric stenosis, and from an upper lip cut. He had received multiple units of cryoprecipitate from the first month of life until the present admission. Work-up demonstrated prolonged bleeding time and prolonged PTT, normal prothrombin time, thrombin time, and platelet aggregation with ADP, epinephrine and collagen. Ten days after cessation of factor VIII replacement therapy he had undetectable F VIII-AHF, F VIII-Ag of 86% by the Laurell technique and F VIII-RCo of 18%. We could demonstrate a time- dependent inhibitor directed against F VIII-AHF measuring 92 Bethesda units. In mixing studies with normal plasma, little or nor inhibitory activity directed against F VIII-RCo could be identified. The patient’s platelets showed increased ristocetin-induced platelet aggregation (RIPA) at doses as low as 0.5 mg/ml.  There was no evidence of a bleeding disorder in the maternal side of the family. All of the paternal family members studied showed prolonged bleeding times, decreased F VIII-RCo, and increased RIPA with low doses of ristocetin, findings similar to those patients recently characterized as having type IIb vWD. Although other members of the patient’s family had previously received cryoprecipitate replacement therapy, no inhibitors appear to have developed in these persons.  Factor VIII inhibitors in vWD are uncommon, with those reported usually showing little or no inhibition of F VIII-AHF. These findings appear to represent a unique pattern of inhibitor development that may be related to a molecular variant of vWD in this patient.

An Inhibitor Selectively Directed Against Factor VIII-AHF In A Patient With Von Willebrand’S Disease (vWD)