A family with dysfibrinogenemia is described. The abnormal fibrinogen occurred in
three successive generations indicating a dominant hereditary pattern. Thrombin and
reptilase times were about twice the normal value. This was shown to be caused by
a polymerization defect, fibrinopeptide release being normal. Platelet aggregation
was undisturbed, indicating normal platelet-fibrinogen binding. The bleeding time
was normal and there was no bleeding tendency. However, an obscure recurrent pulmonary
ailment may, or may not, be related to the dysfibrinogenemia. The abnormal fibrinogen
was tentatively termed Oslo IV.
Keywords
Dysfibrinogenemia - Polymerization defect - Inheritable
