Summary
Hageman factor deficiency was found in a 23-year-old man with a considerably prolonged clotting time, but no haemorrhagic diathesis. The investigations showed pronounced heparin sensitivity, delayed and reduced thromboplastin generation, delayed, but marked thrombin generation, and a prolonged partial thromboplastin time. The defect was completely eliminated by indirect glass activation experiments using plasma with a partial factor VIII, IX, and PTA deficiency as well as a p-p of l% (after phenylindanedione). Normal platelets almost completely corrected defect, whereas the patient’s own platelets could not exert this activity until they had been suspended in normal plasma. Erythrocyte haemolysate in a weak concentration completely eliminated the coagulation defect.
