PEDIATRICS Vol. 75 No. 2 February 1985, pp. 280-283
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Cytomegalovirus-Associated Hemophagocytic Syndrome

ELIZABETH H. DANISH MD1, BEVERLY B. DAHMS MD1, and MARY L. KUMAR MD1

1 Divisions of Hematology and Infectious Disease, Department of Pediatrics, Cleveland Metropolitan General Hospital and Institute of Pathology, Case Western Reserve University, Cleveland

Virus-associated hemophagocytic syndrome, first described by Risdall and co-workers in 1979,1 is a rare histiocytic proliferative syndrome characterzed by fever, hepatosplenomegaly, pancytopenia, and erythrophagocytosis by histiocytes that appear benign by histologic criteria. The clinical course and pathologic findings may be identical with another histiocytic disorder, familial erythrophagocytic lymphohistiocytosis, which occurs predominantly in infants. Diagnosis of virus-associated hemophagocytic syndrome depends entirely on evidence of concurrent viral infection, usually of the herpes group. Epstein-Barr virus has been associated with this syndrome in the few cases reported in children without underlying disease, whereas cytomegalovirus (CMV) has been implicated in immunosuppressed patients. We report a case of fatal CMV-associated hemophagocytic syndrome which occurred in a previously healthy infant.