SEVERE HYPOFIBRINOGENEMIA ASSOCIATED WITH BILATERAL ISCHEMIC NECROSIS OF TOES AND FINGERS

Severe hypofibrinogenemia was found in an Algerian woman who, since the age of 37 years, suffered three different episodes of ischemic necrosis of the toes and fingers leading to amputation of the toes and surgical removal of necrotic tissue (necretomy). No anti-fibrinogen antibody was present. The deficiency appeared to be due to severe congenital hypofibrinogenemia since the fibrinogen level remained at the same low level over a long period, without any abnormality of other coagulation proteins. The thrombotic events may be explained by the increased thrombin generation observed in the patient's plasma, due to the lack of thrombin adsorption onto a fibrin net.