Congenital afibrinogenaemia and successful pregnancy outcome. Case report

The successful outcome of pregnancy in a 25year-old woman with congenital afibrinogenaemia is discussed. She had been found to have afibrinogenaemia soon after birth, following investigation for severe bleeding from her cord stump. She frequently suffered with severe bruising, heavy menstruation and excessive blceding after dental treatment. She had had an avcrage of two to three fibrinogen transfusions a year at the time of acute bleeding episodes such as following tooth extraction, appendicectomy or for a heavy period. Her first pregnancy in August 1988 miscarried at 7 weeks gestation with excessive bleeding. Her fibrinogen level was undetectable. She was transfused with fibrinogen prior to the evacuation of retained products of conception. Her second pregnancy followed soon after (March 1989), and was complicated with multiple episodes of bleeding from the 5th to 13th week, during which time she received intermittent fibrinogen transfusions. Repeated weekly 5 gm fibrinogen transfusions from the 13th week served to halt the bleeding, but premature separation of the placenta a t 27 weeks gestation resulted in preterm labour and the birth of a live 610 g baby, who died shortly afterwards. Her preand post-fibrinogen transfusion levels during this pregnancy varied between 1 and 100mg/dl and the level was 0.9 mg/dl on the day of the placental abruption. After this tragic episode it was decided she should have prophylactic fibrinogen transfusions during the next pregnancy. To avoid the incon-