A Case of Hemophilia B Diagnosed in a Patient Who Presented with Repeated Post-Tonsillectomy Hemorrhage

Abstract

Hemophilia B is an X-linked disease characterized by decreased in blood level of coagulation factor IX activity. Mild to moderate hemophilia is often diagnosed only after surgery or trauma. We encountered a case of hemophilia B, in which the diagnosis was made after the patient presented with recurrent postoperative hemorrhage. The patient was a 4-year-old boy without any remarkable past medical history of bleeding disorders. He was diagnosed as having severe obstructive sleep apnea, and underwent adenotonsillectomy under general anesthesia. Postoperative hemorrhage occurred on day 2 and day 6 after surgery, and procedures for hemostasis were required on both days. He exhibited recurrent hemorrhage and showed a prolonged activated partial thromboplastin time of 62.3 s; therefore, we suspected coagulopathy. Further examination revealed a reduced blood coagulation factor IX activity level, and the patient was diagnosed as having moderate hemophilia B. This case indicates the importance of preoperative screening for coagulation defects, especially before tonsillectomy, as post-tonsillectomy bleeding can be fatal.