Previous results, presented in abstract form, indicate that replacement of thromboplastin with a mixture of phospholipid and truncated soluble tissue factor apoprotein results in a coagulation assay that can directly measure plasma factor VIIa levels without interference from zymogen factor VII (Atherosclerosis Thromb 11:1544a, 1991 [abstr]). We have exploited the specificity and sensitivity of...

This review summarizes current data on the pathomechanisms and new genetic findings of combined factor V and VIII deficiency (CF5F8D). Congenital haemorrhagic disorders characterized by deficiency of two clotting factors comprise an interesting group. Among dual coagulation disorders, CF5F8D is the most common type. For the first time combined factor V and VIII deficiency (F5F8D) was reported b...

Since dialysis of human factor VIII against buffers of low ionic strength yielded two distinct components, and since the factor VIII fraction isolated from normal plasma showed von Willebrand factor activity as defined by the corrective effect on abnormal platelet retention and ristocetin aggregation in von Willebrand's disease, the present studies were performed to determine if the correcting ...

normal VIII-related protein, as described by Holmberg and Nilsson (1973). The three patients to whom cryoprecipitate was given all showed a postinfusion correction of Ristocetin aggregation, although the response of factor VIII and VIII-related protein varied in each case. To the standard definition of classical von Willebrand's disease, it is now possible to add abnormal Ristocetin-induced agg...

Southern blotting was performed with cDNA probes for the human von Willebrand factor (vWF) gene on six patients with severe type 111 von Willebrand’s disease (vWD). A partial deletion in the 3‘ end of the vWF gene was demonstrated in one individual whose parents were related and who had an alloantibody inhibitor to vWF. A resulting novel 2.0-kilobase (kb) EcoRl fragment was used for carrier det...

Human factor VIll-anti-factor VIII comThe extent of dissociation was measured plexes were formed in vitro in slight antiby the recovery of anti-factor VIII activity. gen excess, using plasma of hemophiliacs Increasing resistance of complexes towho were found to have antibodies neuwards dissociation was observed in the tralizing AHF activity. These complexes, plasma of the patients whose titer o...

normal VIII-related protein, as described by Holmberg and Nilsson (1973). The three patients to whom cryoprecipitate was given all showed a postinfusion correction of Ristocetin aggregation, although the response of factor VIII and VIII-related protein varied in each case. To the standard definition of classical von Willebrand's disease, it is now possible to add abnormal Ristocetin-induced agg...