INCREASED THROMBOTIC TENDENCY IN A FAMILY WITH HEREDITARY ANGIONEUROTIC EDEMA ( HANE )

Biochemical hallmark of HANE is a reduction of C1-inhibitor. We observed a family with type II disease ( non-functional protein ), in which 3 of 6 affected members had arterial thromboembolic events at young ages. For evaluation of alterations in the hemostatic system analysis included: fibrinogen, FVII, FVIII, FIX, FXI, FXII, prekallikrein PK, antithrombin III ATIII, protein C PC, α₂ antiplasmin α₂AP, cl α₂ macroglobulin α₂ MG, plasminogen activator inhibitor PAI, plasminogen PG, euglobulin clot lysis time ECLT and tissue plasminogen activator tPA at baseline and after venous occlusion. The results are shown in part in the table:

There is evidence of nearly no response to venous occlusion in 2 and a diminished response in 1 out of 4 patients.

We conclude, that the increased thrombotic tendency in this family is related to the increased potential of prephase coagulation factors and impaired fibrinolytic response to venous occlusion concomitantly with the reduction of the main inhibitor of the contact activation system.