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.
