Abstract
BACKGROUND: Hemophilia is an X-linked recessive inherited bleeding disorder caused by genetic defects in specific proteins involved in blood coagulation. It is classified into two main types: hemophilia A and hemophilia B. The primary clinical characteristic of hemophilia is prolonged spontaneous and/or traumatic bleeding, which most commonly occurs within the musculoskeletal system. However, spontaneous splenic rupture in adults with hemophilia A has been reported infrequently. This rare but life-threatening condition necessitates prompt diagnosis and intervention. This study aims to evaluate the safety and efficacy of splenic artery embolization (SAE) in patients with hemophilia A who present with spontaneous splenic rupture and maintain hemodynamic stability. CASE DESCRIPTION: This case report describes a 20-year-old male with hemophilia A [factor VIII (FVIII) activity at 5%] who presented with intermittent epigastric distension and discomfort with a 3-day history. Imaging studies confirmed the presence of splenic rupture with hemoperitoneum. Emergency SAE was performed, successfully achieving hemostasis. Both preoperatively and postoperatively, the patient received FVIII replacement therapy along with other blood products. As a result, the patient's FVIII level increased to 31%, hemoglobin stabilized at 100 g/L, and the patient was discharged after 1 week. CONCLUSIONS: This case underscores the efficacy of SAE as a minimally invasive and effective treatment for spontaneous splenic rupture in patients with hemophilia A, particularly in hemodynamically stable individuals.