Functional deficiency of protein C associated with mesenteric venous thrombosis and splenic infarction.

A 16-year-old girl had abdominal pain, hematemesis, and fever. Radiographic evaluation showed her to have an infarcted spleen and jejunum. Pathologic description revealed thrombi involving the vessels of both organs. A hypercoagulation evaluation of the patient and her family resulted in the diagnosis of hereditary protein C activity deficiency. She is unusual both in the diagnosis of protein C activity deficiency and in her appearance with mesenteric and splenic venous thrombosis. This appearance has not been previously reported in the literature and should be considered in the differential diagnosis of any thrombotic event, even in the face of a negative family history.