Rieger C H, Lustig J V, Justman R A, Rothberg R M
Eur J Pediatr. 1976 Dec 9;124(1):51-6. doi: 10.1007/BF00452414.
Three children with chronic lymphoid hyperplasia and hypergamma-globulinemia are presented. They had normal numbers of circulating T-cells and normal in vitro lymphocyte responses to phytohemagglutinin and specific mitogens. The number of cells staining for surface immunoglobulins was decreased in two patients but increased in one patient with chronic lymphocytosis which is still present 5 years after his initial hospitalization. Lymphocytes without detectable markers ("Null cells") were increased in all three patients. Levels of antibody titers to common antigens were normal. Lymph node biopsies showed nonspecific hyperplasia of the B-cell areas. The combination of normal immune responses with lymphoid hyperplasia and with a chronic lymphocytosis in one of the children suggests that this syndrome may be due to a defect in the regulation rather than the initiation of immune responses.
本文报告了3例患有慢性淋巴细胞增生和高γ球蛋白血症的儿童。他们循环T细胞数量正常,对植物血凝素和特异性有丝分裂原的体外淋巴细胞反应正常。两名患者表面免疫球蛋白染色的细胞数量减少,但一名慢性淋巴细胞增多症患者的该细胞数量增加,该患者首次住院5年后仍存在慢性淋巴细胞增多症。所有3例患者中无可检测标志物的淋巴细胞(“裸细胞”)均增多。对常见抗原的抗体滴度水平正常。淋巴结活检显示B细胞区非特异性增生。其中一名儿童出现正常免疫反应伴淋巴细胞增生和慢性淋巴细胞增多症,这表明该综合征可能是由于免疫反应调节缺陷而非启动缺陷所致。