Synthesis of Ahf in Von Willebrand's Disease.

BLOOD, VOL. 23, No. 2 (FEBRUARY), 1964 By Jitssic & H. Li wis A HEMORRAGIC DISEASE, first described by von Willebrand in 1926’ in a large family living on the Aland Islands, has gone through a fascinating diagnostic metamorphosis. Initially it was thought to be due to a platelet abnormality,2’3 later to a vascular abnormality;4’5 more recently, to lack of a plasma factor( s ) .#{176} ‘ This changing pathogenetic view reflects not only increasing diagnostic skill but also inclusion in some publications of patients with varying hemostatic defects. In 1957, Nilsson and co-workers7 studied 15 members of the original family on the Aland Islands and found mild AHF (factor VIII ) deficiency in all bleeders and prolonged bleeding times in those with moderate to severe hemorrhagic symptoms. Other tests, including those of platelet function, were normal. In one patient the prolonged bleeding time was corrected by an infusion of plasma fraction 1-0. Thus it seemed possible to re-define von Willebrand’s disease as a hemorrhagic disorder, usually familial, characterized by a prolonged bleeding time and a low level of AHF. With this definition in mind, descriptions of many similar cases can be found in the literature including those in which the disease has been called “vascular hemophilia,” “pseudohemophilia B” and “angiohemophilia A.” The patient presented herewith appears to fall within the definition of von Willebrand’s disease although she lacks a positive family history. Of particular importance is the apparent ability of this patient to synthesize her own AHF when infused with normal or hemophulic plasma.