Hepatic manifestations of hemophagocytic syndrome: A study of 30 cases

OBJECTIVE: Hemophagocytic syndrome has been defined as the combination of a proliferation of cytologically benign, actively phagocytic macrophages in bone marrow, spleen, or lymph nodes in association with fever, cytopenia, s plenomegaly, and hypertriglyceridemia. Hepatic dysfunction is often present but the nature of the hepatic lesions and related manifestations have not been fully characterized. The aim of this study was to ascertain the featur es of hepatic involvement in hemophagocytic syndrome. METHODS: Thirty patients with hemophagocytic syndrome were retrospectively studied. Inclusion criteria included: 1) bone marrow with hemophagocytic hi stiocytosis, 2) clinical or biological signs of hepatic involvement, and 3) available liver specimen. RESULTS: The association of fever, jaundice, and hepatomegaly or splenomega ly was present in 50% of the patients. Median value for serum alanine trans aminase activity was five times the upper limit of normal values (range, 0. 3-125), for serum alkaline phosphatase activity 2.7 upper limit Of normal v alues (range, 0.2-47.7), for total bilirubin 136 mu mol/L (range, 4-681 mu mol/L), and for factor V 70% (range, 19-145%). Sinusoidal dilatation with h emophagocytic histiocytosis were found in the biopsy specimen in all patien ts. An underlying condition potentially responsible for altered immune func tion (lymphoma, leukemia, liver transplantation) was identified in 29 patie nts. Liver biopsy was diagnostic for the underlying condition in 15 patient s (including eight cases with nonspecific bone marrow biopsy findings). Hig h serum bilirubin, elevated serum alkaline phosphatase activity, low factor V level, and lack of treatment fur the underlying disease were associated with a poor prognosis. CONCLUSIONS: Hemophagocytic syndrome should be suspected in immunodeficient patients with fever, jaundice, and hepatosplenomegaly. Hepatic lesions an characterized by nonspecific sinusoidal dilatation with hemophagocytic hist iocytosis and in 50% of the patients by alterations specific to the underly ing condition. Liver biopsy is a useful diagnostic procedure in patients wi th this clinical presentation. (C) 2001 by Am. Cell. of Gastroenterology.