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Thromb Haemost 1961; 05(02): 187-200
DOI: 10.1055/s-0038-1654919
Originalarbeiten – Original Article – Travaux Originaux
Schattauer GmbH

Coagulation Studies in a Case of Hageman Trait

E. A Loeliger
1   Clinic for Internal Medicine (Director: Prof. J. Mulder, M.D.) University Hospital, Leyden, The Netherlands
,
A Hensen
1   Clinic for Internal Medicine (Director: Prof. J. Mulder, M.D.) University Hospital, Leyden, The Netherlands
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Publikationsdatum:
21. Juni 2018 (online)

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Summary

After a brief review of the data concerning the cases of Hageman trait hitherto reported in the literature, a patient with severe Hageman factor (HF) “deficiency” (HF-activity below 0.05% of normal) is described. The patient has no haemorrhagic diathesis.

In vitro, intrinsic coagulability and fibrinolytic activity are grossly disturbed. On the basis of a diminished fibrinolytic activity in vivo, the rather high activity of several coagulation factors in patient’s plasma could be explained.

Thromboplastin formation, once initiated, is normal as to rate; the amount of thromboplastin formed is possibly slightly diminished. Prothrombin consumption is normal. These findings are in agreement with a normal rate ot clot formation, as measured by means of thrombelastography.

Normal HF seems to be an initiator of blood coagulation only and not an activator or a substrate involved in thromboplastin formation.

The weak anticoagulant property of Hageman trait plasma, described by several authors, is not necessarily due to an inhibitor; it can be explained by the assumption that, in Hageman trait, HF shows normal glass adsorbability and only a very deficient glass activation.

 

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