Increased platelet retention in familial recurrent thrombotic thrombocytopenic purpura

We studied two brothers with recurrent thrombotic thrombocytopenic purpura (TTP). Platelet retention, measured with a modified Adeplat S glass-bead test, was found to be increased during acute episodes of TTP and during remissions. Values of platelet retention ranged between 57 to 95% (normal range 16 to 34%). The continually elevated values enabled us to investigate which fraction of the patients' blood was responsible for the increased platelet retention and to evaluate the effect of different treatments on this parameter. We found that the patients' plasma increased the retention of normal platelets and of platelets taken from a patient with von Willebrand's disease type III. This activity was located in the cryoprecipitate fraction of plasma. Unusually large von Willebrand factor (vWF) multimers were demonstrated in both children during remission and decreased during relapse. Both fresh frozen plasma (FFP) and a commercial factor VIII/vWF concentrate reduced platelet retention when tested during remission. Treatment of both siblings with FFP or factor VIII/vWF concentrate was beneficial during recurrences. We conclude that the elevated platelet retention is due to a factor in the cryoprecipitate of the childrens' plasma, and that both FFP and factor VIII/vWF concentrate are effective in decreasing platelet retention.