Spontaneous splenic rupture in a patient with factor XIII deficiency: A case report

Spontaneous splenic rupture is a rare, life-threatening event, infrequently associated with congenital factor XIII deficiency, a rare coagulation disorder. We present a case of an 18-year-old female with known factor XIII deficiency who presented with acute abdominal pain and was diagnosed with spontaneous splenic rupture. The patient underwent splenectomy due to hemodynamic instability and massive hemorrhage. Postoperative management included factor XIII replacement and standard postsplenectomy prophylaxis. Spontaneous splenic rupture in the setting of factor XIII deficiency is exceedingly rare, with only a limited number of cases reported in the literature. This case highlights the importance of considering coagulation disorders in the differential diagnosis of atraumatic splenic rupture. Prompt diagnosis and multidisciplinary management, including surgical intervention when necessary, are critical for optimizing patient outcomes. This case underscores that factor XIII deficiency can lead to spontaneous life-threatening hemorrhage. Clinicians should maintain a high index of suspicion for this pathology in patients with known coagulopathies, and timely intervention is essential to improve patient outcomes. The importance of this approach is to quickly treat it when needed because is very deadly.