THE ROLES OF INTERLEUKINS IN THE PATHOGENESIS OF HEMPHAGOCYTIC SYNDROME

在项目期间(1994.4.1-1996.3.31)。我们对43例本病患者的外周血单核细胞、骨髓细胞、淋巴结细胞、脾细胞和肝细胞进行了吞噬血细胞综合征(HPS)的研究。流式细胞仪和免疫细胞化学分析细胞表型。通过染色体分析、T细胞受体和免疫球蛋白基因重排的Southern分析以及EB病毒基因组分析来研究克隆性。采用ELISA法或免疫放射分析法检测活动期和非活动期患者血清中各种细胞因子(干扰素-g、IL-6、可溶性IL-2受体、IL-12)水平，结果显示EB病毒感染16例(38%)。16例中T细胞系2例，NK细胞系6例，T细胞或NK细胞系4例。在EB病毒感染的16例HPS患者中，11例(69%)死于疾病，表明该病预后较差。活动期HPS患者血清中干扰素-γ、IL-6、可溶性IL-2受体水平均极高，与EB病毒感染无关。而EB病毒感染引起的感染性单核细胞增多症(IM)患者血清中IL-12水平低于3.0U/ml，而29例HPS患者和4例IM患者血清中IL-12水平均低于3.0U/ml。5例HPS患者血清IL12水平升高(1.4~22.5 ng/ml)。这可能表明IL-12参与了某组EB病毒相关性HPS的发病。

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.