THE ROLES OF INTERLEUKINS IN THE PATHOGENESIS OF HEMPHAGOCYTIC SYNDROME

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During the term of project (1994.4.1-1996.3.31). we have investigated hemophagocytic syndrome (HPS) using samples (peripheral blood mononuclear cells, bone marrow cells, lymphnode cells, spleen cells, and liver cells) obtained from 43 cases of this disease. Cellular phenotype was analyzed by flow cytometer and immunocytochemistry. Clonality was studied by chromosomal analysis, and Southern analysis of T cell receptor and immunoglobulin gene rerrangenment, and EB viral genome. Serum levels of various kinds of cytokines (IFN-g, IL-6, soluble IL2 receptor, IL-12) at active and inactive phase were measured by ELIZA or immunoradioassay.The results showed that EB viral involvement was demonstrated in 16 cases (38%). Of the 16 cases, two cases were T cell lineage, 6 cases were NK cell lineage, and 4 cases were T cell or NK cell lineage. Because od shortage of samples lineage was not identified in 4 cases, and the rest one case was reactive HPS.Of the EB virus associated 16 HPS cases, 11 cases were died of disease (69%), which clearly showed that this type of disease has quite poor prognosis. Serum IFN-g.IL-6, soluble IL-2 receptor levels in active HPS were extremely high regardless of EB viral involvement. However, IFN-g was especially high in EB virus associated HPS (9.2-330U/ml, normal<0.1U/ml), while its serum levels in infections mononucleosis (IM).which is also caused by EB viral infection, was below 3.0U/ml. We also analyzed serum IL12 levels in 29 cases of HPS and 4 cases of IM by establishing ELIZA measuring system. IL12 level was elevated in 5 case of HPS (1.4-22.5ng/ml). This may indicates that IL-12 is involved in a certain group of EB virus associated HPS.