IMMUNOMODULATION TREATMENT FOR CHILDHOOD VIRUS-ASSOCIATED HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS

The Epstein-Barr virus (EBV), or human herpesvirus-6 (HHV-6) associate d haemophagocytic lymphohistiocytosis, has been found prevalent in Tai wan; it affects previously healthy children and is always fatal when t reated only supportively. Recognition of the underlying pathogenesis f or this disease prompted adoption of an immunomodulatory regimen of in travenous immunoglobulin (IVIG) and/or etoposide on 17 such patients t reated between 1990 and 1993. Remarkable improvement in patients' prog noses was demonstrated. Eight patients are still alive with a median f ollow-up of 1 year and 2 months post-treatment. Both MG and etoposide had positive immunomodulation effects such as alleviation of fever and normalization of haematological and hepatic parameters. Sustained com plete response was obtained in two of nine cases of EBV-associated dis eases treated with MG only, EBV transcripts became undetectable after etoposide and/or IVIG treatment without antiviral agents. Etoposide gi ven by split-dose schedule appeared to be superior to conventional thr ee-consecutive-days schedule for both remission induction and disease- free survival. Our preliminary trial apparently provides a promising i mprovement in the treatment of this previously fatal disease, IVIG or etoposide is effective in reversing the process of lymphohistiocytic d ysregulation resulting from virus infection of immune cells in this sy ndrome and probably helps hosts to control active virus replication in certain cases, through immunomodulation.