Malignant histiocytosis in a patient presenting with hepatic dysfunction and peliosis hepatis.

Abstract

In this article, we report the case of a 36-yr-old patient presenting with manifestations of portal hypertension, hepatic dysfunction, and fever who proved to have peliosis hepatis on liver biopsy. A thorough work-up revealed no obvious etiology. At autopsy, malignant histiocytosis of the liver and bone marrow was diagnosed. This case represents the first report of the association of peliosis hepatis with this rare histiocytic neoplasm and exemplifies the need for persistence in the search for malignancy, particularly hematological malignancy, in the patient with unexplained peliosis. The clinical similarity of peliosis hepatis associated with hematological malignancy and bacillary peliosis is also discussed.