Hemophilia B with Spontaneous Itraperitoneal Bleeding: A Case Report

Abstract

Hemophilia B is a congenital bleeding disorder due to the decrease of the clotting factor IX activity. Its common manifestation in severe form is spontaneous hemarthrosisparticularly at the weight-bearing joints. Herein we report an 18-year old Thai man who was diagnosed as severe hemophilia B since two months of age and he continually has had one or two episodes of hemarthrosses a year. He repeatedly has an isolated prolongation of aPTT and his factor IX activity at the steady state is around 0.8% while factor VIII activity is normal. At this admission, he spontaneously develops acute abdominal pain for one day due to the intraperitoneal bleeding, the unusual site of spontaneous bleeding manifestation of hemophilia B. He has no previous abdominal trauma. The computerized tomography of the abdomen confirms lots of hemoperitoneum and the thickening of bowel wall that is supposed to be the original site of bleeding whereas the liver and spleen appear normal. His hematocrit is found to be 18% and he is treated with many units of packed red blood cell transfusion as well as factor IX concentrate 5,000 unit every day. His abdominal symptom is gradually improved and he can be discharged within seven days without any operation.