[Post partum acquired hemophilia A with fatal outcome: a case report]. / À propos d'un cas d'hémophilie A acquise du post partum à évolution fatale.

ABSTRACT

We report the case of a 19-year-old women who developed on post partum day 1 a large hematoma in the vaginal wall. A surgical intervention for evacuating the hematoma was complicated with profuse bleeding of the genital and abdominal cavities. Postoperative coagulation studies revealed a prolonged activated partial thromboplastin time (87/30 s; ratio 2.9), a decreased factor VIII level at 7% and the presence of an anti-factor VIII inhibitor in a titer of 64 Bethesda Unit (BU). Due to the active bleeding, the patient received Novoseven(®) (recombinant factor VIIa 2 doses on post partum day 3 and 1 dose on day 6) and Feiba(®) (activated prothrombin complex concentrates 1 dose on post partum day 8). These few administrated doses were not enough efficacious to achieve a continuous control of the bleeding. Immunosuppressive treatment used prednisone alone for 3 days, then combined to cyclophosphamide. The outcome was marked by the aggravation of the bleeding and an increase in the inhibitor level (132 BU). Finally, the patient died on post partum day 8 following an hemorrhagic shock.