[Dysfibrinogenemia and thrombosis. A case report].
BACKGROUND
Congenital dysfibrinogenemia is a functional disorder of the fibrinogen that represents a rare cause of thrombophilia.


OBJECTIVE
To report a Tunisian case of the association dysfibrinogenemia and thrombosis.


METHODS
A woman with inherited dysfibrinogenemia associated with mild tendency to bleeding experienced a deep vein thrombosis of the lower-extremity at 26 years of age and a fatal pulmonary embolism a few years later. Paradoxically coagulation function of fibrinogen was markedly altered in vitro with a significantly prolonged prothrombin time, activated partial thromboplastin time and thrombin time, a functional fibrinogen level that was undetected and a severely impaired fibrin polymerisation. The thromboembolic events in the patient could be related to dysfibrinogenemia since the main causes of thrombophilia were excluded.


CONCLUSIONS
Although it is rare, this cause of thrombophilia must not be misdiagnosed, systematic measuring of prothrombin time, activated partial thromboplastin time and functional fibrinogen might be helpful.
Kraiem
Imen
I
Laboratoire d'Hématologie, Institut Pasteur de Tunis, Tunisie.
Guermazi
Sami
S
Ben Abid
Héla
H
Meddeb
Balkis
B
D000328:Adult; D000347:Afibrinogenemia / Q000150:complications*; D017809:Fatal Outcome; D005260:Female; D015241:Fibrinogens, Abnormal / Q000235:genetics; D006801:Humans; D011655:Pulmonary Embolism / Q000209:etiology; D019851:Thrombophilia / Q000150:complications; D020246:Venous Thrombosis / Q000097:blood*
D015241:Fibrinogens, Abnormal