Budd–Chiari Syndrome in an Afibrinogenemic Patient: A Paradoxical Complication

Abstract

Qualitative and quantitative abnormalities of fibrinogen can result in hemostatic disorders (1). Congenital afibrinogenemia is a rare coagulation disorder transmitted as an autosomal recessive trait (2). The incidence of afibrinogenemia is 1–2 cases per million population (3). It is more common in populations with a high rate of consanguinity (3). Symptoms of afibrinogenemia vary from mild hemorrhagic events to severe bleeding episodes, though thrombotic events are very rare. We report an afibrinogenemic patient who presented with hepatic venous thrombosis at 16 years old. Evaluation of all hemostatic parameters and coagulation factors revealed no other pathology except afibrinogenemia. Paradoxically, afibrinogenemia may be a risk factor for vascular thrombotic events like Budd–Chiari Syndrome (BCS).