[Congenital dysfibrinogenaemia. Two new cases (author's transl)]

Congenital dysfibrinogenaemia is a rare disorder related to an abnormality in the behaviour of the fibrinogen molecule. The diagnosis may be made with the aid of simple tests, including prothrombin'time, even though this examination may be normal in a small number of cases. Two new cases are reported here. Neither of the two women involved had a past history of severe haemorrhage, the diagnosis being made at the time of routine coagulation studies. According to the international nomenclature, we suggest the names fibrinogen Paris IV and fibrinogen Buenos-Aires II. In order to avoid missing the diagnosis, fibrinogen should be estimated by different methods in the presence of hypofibrinaemia. There is disagreement between fibrinogen levels estimated by the thrombin chronometric method, in general low, and levels obtained by gravimetric and immunological methods, usually normal.