Objective: To explore the clinical and pathologic features as well as prognosis of systemic EBV-positive T-cell lymphoma in children.
Methods: The clinical data including clinical manifestation, pathologic changes and treatment in 16 patients with children's systemic EBV-positive T-cell lymphoma were analyzed retrospectively, and follow-up of patients were carried out.
Results: The 16 cases included 12 males and 4 females with median age of 3.3 years old. It was demonstrated that the clinical and pathological features of the children's systemic EBV-positive T-cell lymphoma were as followed fever, hepatosplenomegaly, cytopenia, lymphadenopathy, and hemophagocytosis in bone marrow or organ. Histologically, the structures of lymph node was normal, partially or completely destoryed. The paracortical zone was expanded with prominent infiltration of small to medium-sized atypical lymphocytes. The major immunophenotypic characteristics were as follows: (1) Almost all biopsies exhibited prominent T cell proliferation. (2) CD3 was expressed in 16 patients (100%, 16/16), CD4 in 5 patients (31.3%, 5/16)，CD5 in 13 patients (81.3%, 13/16)，CD7 was expressed in 11 patients (68.8%, 11/16)，CD8 in 15 patients (93.8%, 15/16)，CD4 and CD8 were expressed in 5 patients (31.3%, 5/16)，CD4 and CD8 double-negative in patients (6.3%, 1/16)，16 patients were CD56 negative (100%, 16/16). (3) TCR gene cloning rearrangement in 16 patients (93.8%, 15/16). (4) EBV-EBER was expressed in 16 patients (100%, 16/16). 11 out of 16 cases died, 1 cese failed to be followed up, 1 case relapsed，and 3 cases survived, reseptively. The media survival time was 4 months.
Conclusion: Systemic EBV-positive T-cell lymphoma predominantly occurred in childhood and early teen-age, and lacks specific clinic features, usually combined with hemophagocytic syndrome. The confirmed diagnosis requires comprehensive analysis of clinical manifestation, pathomorphology, immunohistochemical detection, EBV-EBER insite hybridization, and TCR gene test. The overall prognosis of the disease is poor and the fatality rate is high.
题目: 儿童系统性EB病毒阳性的T细胞淋巴瘤临床病理分析.
目的: 探讨儿童系统性EB+ T细胞淋巴瘤临床特征、病理学特点及预后.
方法: 对16例儿童系统性EB+ T细胞淋巴瘤病例进行回顾性研究，并进行随访.
结果: 男12例，女4例，中位年龄3.3（1-17）岁，临床上以发热、肝脾肿大、血细胞减少、淋巴结肿大、骨髓或器官可见噬血现象为主要表现。组织病理特点：淋巴结结构可正常、部分或完全破坏，淋巴结副皮质区增宽，瘤细胞弥漫增生，细胞中等大或体积偏小，轻度异型或显著异型性。免疫表型特点：增生细胞大多数为T细胞，CD3+ 100%（16/16），CD4+ 31.3%（5/16），CD5+ 81.3%（13/16），CD7+ 68.8%（11/16），CD8+ 93.8%（15/16），CD4+CD8+ 31.3%（5/16），CD4-CD8- 6.3%（1/16），CD56- 100%（16/16）。EBER原位杂交阳性100%（16/16），TCR呈单克隆性基因重排93.8%（15/16）。死亡11例，失访1例，复发1例，生存3例，中位生存时间4个月.
结论: 儿童系统性EB+ T细胞淋巴瘤好发于儿童和年轻成人，在婴幼儿发病亦不少见，临床表现缺乏特异性，常合并噬血细胞综合征，确诊需结合临床表现、病理形态、免疫组织化学、EBV-EBER原位杂交及TCR基因检测等综合分析。总体预后不良，病死率高.