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Thromb Haemost 1962; 07(02): 249-257
DOI: 10.1055/s-0038-1655473
Originalarbeiten — Original Articles — Travaux Originaux
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Hageman Factor Deficiency. (Demonstrated by Indirect Glass Activation)

J Gormsen
1   Laboratory, Medical Department F (Head: Professor F. Wulff, M.D.), the Copenhagen County Hospital in Gentofte, Hellerup, Denmark
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Publication Date:
21 June 2018 (online)

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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.

 

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