Nezelof C, Maupas C, Griscelli C
Unité d'Immunologie et d'Hématologie Pédiatriques (INSERM U 132), Hôpital Necker Enfants Malades, Paris, France.
Pediatr Pathol. 1989;9(1):57-71. doi: 10.3109/15513818909022332.
Three cases of a syndrome featuring massive splenomegaly, gross generalized lymphadenopathy, and moderate hepatomegaly are reported. Spleen weights ranged from 800 to 2400 g. Gradual depletion of lymphoid germinal centers, and prominent infiltration of the splenic and lymph node cords with plasma cells, immunoblasts and actively dividing B cells were the most distinctive histological features. The liver in two cases showed portal infiltrates. A marked hypergammaglobulinemia, a decrease in blood cholesterol level and hematological abnormalities related to hypersplenism were observed. The condition begins early in life and runs a chronic course, of up to 25 years. There was a family history in only one instance. Since there was no generalized immunodeficiency nor local depletion of T cells or dendritic reticulum cells, a failure in the local regulation of the immune response and possible cytokine production is postulated. This condition underlines the pivotal role of the local organization of the germinal centers in cellular cooperation and in the carrying out and regulation of the immune response.
报告了3例具有巨脾、全身淋巴结肿大和中度肝肿大特征的综合征病例。脾脏重量在800至2400克之间。淋巴生发中心逐渐耗竭，脾和淋巴结索中浆细胞、免疫母细胞及活跃增殖的B细胞显著浸润是最具特征性的组织学表现。2例患者的肝脏出现门脉浸润。观察到显著的高球蛋白血症、血胆固醇水平降低以及与脾功能亢进相关的血液学异常。该病起病于早年，病程长达25年。仅1例有家族史。由于不存在全身性免疫缺陷，也没有T细胞或树突状网状细胞的局部耗竭，推测是免疫反应的局部调节以及可能的细胞因子产生出现了故障。这种情况突出了生发中心局部组织在细胞协作以及免疫反应的执行和调节中的关键作用。
