[Successful treatment with intermittent administration of etoposide for an adult case with recurrent hemophagocytic syndrome, developed in association with chronic EB virus infection related lymphoid hyperplasia in the small intestine].
A 28-year-old man complaining of melena accompanied by high grade fever was previously operated on for two small ulcerative, infiltrative lesions of the small bowel at another hospital in April, 1992. In the resected small intestines an immunohistochemical study was positive for EBV encoded RNA (EBER). It is assumed that Epstein-Barr (EB) virus infection has contributed to the small bowel lesions. In March, 1993, he was diagnosed as having chronic EB virus infection. In October, 1993, he was transferred to our hospital for further examination of high grade fever, rapidly worsening thrombocytemia and severe liver disfunction. Mature histiocytes with hemophagocytosis were detected in the bone marrow and he was diagnosed as having hemophagocytic syndrome (HPS) due to reactivation of chronic EB virus infection. Although treatment by corticosteroids, acyclovir and gamma globulin was not effective administration of etoposide induced remission of the disease. In December, 1993 and January, 1994 HPS recurred, but etoposide was again effective in alleviating the disease. To prevent recurrence of HPS after we discharge the patient, intermittent administration of small doses of etoposide was started. Since then HPS has not recurred, and the drug was eventually discontinued in November, 1995. Our case suggested that Etoposide could change the fulminant course of EB virus-associated HPS and that it is effective to sustained remission.
Hanai
M
M
First Department of Internal Medicine, Nihon University School of Medicine.
Takei
M
M
Yamazaki
T
T
Morimoto
K
K
Sawada
U
U
Horie
T
T
D000328:Adult; D005047:Etoposide / Q000008:administration & dosage*; D006566:Herpesviridae Infections / Q000150:complications*; D004854:Herpesvirus 4, Human; D015616:Histiocytosis, Non-Langerhans-Cell / Q000188:drug therapy* / Q000209:etiology*; D006801:Humans; D007410:Intestinal Diseases / Q000150:complications*; D007421:Intestine, Small; D008297:Male; D019310:Pseudolymphoma / Q000150:complications*; D014412:Tumor Virus Infections / Q000150:complications*
D005047:Etoposide