Schuster V, Kreth H W, Müller-Hermelink H K, Huppertz H I, Feller A C, Neumann-Haefelin D, Wiegand H, Müller-Lantzsch N
Department of Paediatrics, University of Würzburg, Federal Republic of Germany.
Eur J Pediatr. 1990 Nov;150(1):48-53. doi: 10.1007/BF01959480.
We report on a 30-month-old previously healthy Turkish boy who presented with fever, hepatosplenomegaly and generalized lymphadenopathy. He died 4 months after admission in spite of treatment with steroids, acycloguanosine and cyclophosphamide. Epstein-Barr virus (EBV) DNA was detected in the patient's bone marrow and in a lymph node biopsy. Cells from the lymph node biopsy showed monoclonal rearrangements of immunoglobulin heavy chain genes but no rearrangements of T-cell receptor beta-chain genes or immunoglobulin kappa chain genes. Serological data indicated chronic active EBV infection. There was a slight increase of CD8 positive cells in peripheral blood and a normal response to T-cell mitogens. However, T-cell lines established with interleukin 2 from lymph node biopsy completely failed to kill autologous EBV-transformed B-cells and K 562 target cells. Moreover, in regression tests the patient's peripheral blood mononuclear cells completely failed to limit outgrowth of autologous EBV infected B-cells. We conclude that the patient's selective immuno-deficiency had led to the rapid development of EBV-associated monoclonal lymphoproliferation.
我们报告了一名30个月大、此前健康的土耳其男孩，他出现发热、肝脾肿大和全身淋巴结病。尽管接受了类固醇、阿昔洛韦和环磷酰胺治疗，他在入院4个月后死亡。在患者的骨髓和淋巴结活检中检测到爱泼斯坦-巴尔病毒（EBV）DNA。淋巴结活检的细胞显示免疫球蛋白重链基因的单克隆重排，但未检测到T细胞受体β链基因或免疫球蛋白κ链基因的重排。血清学数据表明存在慢性活动性EBV感染。外周血中CD8阳性细胞略有增加，对T细胞有丝分裂原的反应正常。然而，用白细胞介素2从淋巴结活检建立的T细胞系完全无法杀死自体EBV转化的B细胞和K562靶细胞。此外，在回归试验中，患者的外周血单个核细胞完全无法限制自体EBV感染B细胞的生长。我们得出结论，患者的选择性免疫缺陷导致了EBV相关单克隆淋巴细胞增殖的快速发展。
