Spontaneous splenic rupture in a patient with congenital factor XIII deficiency: A case report and literature review.
Dilek Keskin, Murat Kayır, Tugrul Elverdi
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Case in brief
A 32-year-old man with congenital factor XIII deficiency presented with sudden abdominal pain and hypovolemic shock. Contrast-enhanced abdominal CT showed a large perisplenic hematoma, and surgery confirmed splenic rupture with severe intra-abdominal bleeding. Intensive transfusion and replacement with fresh frozen plasma and cryoprecipitate were given, but splenectomy was required. Histopathology found no underlying splenic disease.
What made this case unusual
The rupture occurred without trauma or an underlying splenic pathology in a patient with congenital factor XIII deficiency, an association rarely reported.
Diagnostic / clinical pearl
In a patient with factor XIII deficiency and acute abdominal pain or shock, consider spontaneous splenic rupture even when there is no trauma history or known splenic disease.