Thrombotic and hemorrhagic presentation of congenital hypo/afibrinogenemia

Congenital fibrinogen disorders are rare in emergency departments (EDs). We describe 2 cases with different presentations; the first was a 27-year-old woman with a known case of congenital hypofibrinogenemia who presented with nausea, vomiting, abdominal pain, and distension. She reported a history of concentrated fibrinogen use (1000 mg/d) for 4 days without visiting any physician. She was diagnosed as having thrombosis of mesenteric and portal veins with intestinal ischemia. The second was a 28-year-old woman with congenital afibrinogenemia who came to our ED with abdominal pain. She was diagnosed as having spontaneous rupture of spleen. Because of various presentations of patients with congenital fibrinogen disorders, both hemorrhagic and thrombotic events should be considered.