Fatal Virus-Associated Hemophagocytic Syndrome in a Young Adult Producing Nontraumatic Splenic Rupture

A 24-year-old man with no previous medical history was admitted to a local hospital with pancytopenia after a recent “viral illness.” During his hospitalization, he developed sudden abdominal distension and hypotension. Surgical exploration of his abdomen revealed a ruptured spleen. The spleen was removed, but the patient did not survive the operation. We investigated this unexpected and unexplained hospital death for any traumatic or iatrogenic injury. The cause of death after review of the clinical history, autopsy, and microscopic sections was virus-associated hemophagocytic syndrome (VAHS). VAHS consists of a generalized histiocytic proliferation and marked hemophagocytosis associated with a systemic viral infection. Clinically it presents as pancytopenia and organomegaly. This recently described entity is often confused with malignant histiocytosis. This is the first case report of VAHS producing nontraumatic splenic rupture, thus adding to the differential diagnosis of spontaneous splenic rupture and sudden natural death.