Spontaneous splenic rupture in a patient with congenital factor XIII deficiency: A case report and literature review.

Spontaneous (atraumatic) splenic rupture is a rare but potentially life-threatening clinical condition. Most reported cases of spontaneous splenic rupture are associated with splenomegaly due to infectious, neoplastic, or inflammatory diseases. Spontaneous rupture in the absence of these conditions is extremely rare. There is often an underlying trauma history. Factor XIII deficiency, a rare autosomal recessive bleeding disorder, is associated with abnormal fibrin stabilization despite normal routine coagulation parameters. Therefore, it can also lead to defects in wound healing. For the same reason, spontaneous splenic rupture has rarely been reported in patients with Factor XIII deficiency. We report a 32-year-old male with congenital Factor XIII deficiency who presented to the emergency room with hypovolemic shock following sudden-onset abdominal pain. The case was complicated by spontaneous splenic rupture and severe intra-abdominal bleeding. Contrast-enhanced abdominal computed tomography revealed a large perisplenic hematoma. Despite intensive blood transfusion and replacement therapy with fresh frozen plasma and cryoprecipitate, the patient required surgical intervention, and a splenectomy was performed. Histopathological examination revealed no underlying splenic pathology. Spontaneous splenic rupture is extremely rare in patients with factor XIII deficiency, but it should be considered in the differential diagnosis in this patient group presenting with an acute abdomen. Suspicion, early recognition, and supportive care with appropriate blood products and factors, along with prompt surgical intervention, are crucial to prevent mortality.