目的：探讨1例儿童系统性EB病毒（Epstein-Barr virus, EBV）阳性T细胞淋巴瘤的临床和病理特征,以加深医师对该病诊断及鉴别方法的认识。方法：综合分析1例儿童系统性EBV阳性T细胞淋巴瘤患者的临床表现、病理形态、免疫表型及基因检测结果,结合近年文献进行探讨。结果：患者为11岁男孩,表现为四肢及躯干皮疹4年余,以螨虫过敏治疗而迁延不愈。患儿近2个月反复发热,出现全身淋巴结肿大和噬血细胞综合征。淋巴结活检示小到中等大的淋巴样细胞弥漫增生;免疫组织化学检测显示,肿瘤细胞CD3（+++）、CD4（+）、TIA-1（+++）、颗粒酶B（+++）、MIB-1 （80%+）;EBV原位杂交示EBER（+++）;T细胞受体（T cell receptor, TCR）基因克隆性重排显示TCRγ克隆性重排。患儿病理诊断为儿童系统性EBV阳性T细胞淋巴瘤。结论：儿童系统性EBV阳性T细胞淋巴瘤是一种少见的EBV感染活化的细胞毒性T细胞克隆性增殖性疾病,临床病程呈暴发性、侵袭性,常伴有噬血细胞综合征,病理需要与侵袭性自然杀伤细胞白血病/淋巴瘤、EBV感染相关淋巴增生性病变及皮肤原发性γδT细胞淋巴瘤鉴别。

Objective: To investigate the clinicopathological characteristics of systemic Epstein-Barr virus (EBV) positive T-cell lymphoma of childhood and improve the understanding of the new diagnostic criteria. Methods: Clinical presentations, pathological characteristics, gene detection results and immunohistochemical phenotyping of biopsy tissue from a case of systemic EBV positive T-cell lymphoma of childhood were analyzed and the relevant literature was reviewed. Result: The 11-year-old boy with prolonged mite allergy treatment and no responsive rash on limbs and torso presented recurrent high fever, generalized lymphadenopathy and hemophagocytic syndrome. Lymph node biopsy revealed diffuse proliferation of small to intermediate neoplastic T cells, which was possibly originated in interfollicular area and positive for CD3（+++）, CD4（+）, TIA-1（+++）, GranzymeB（+++） and MIB-1 (>80% positive). The in situ hybridization of EBV (EBER) was positive （+++） and clonal TCRγ gene rearrangemen was also detected. The diagnosis of systemic EBV positive T-cell lymphoma was thus established. Conclusions: Systemic EBV-positive T-cell lymphoma of childhood is a rare systemic illness characterized by clonal proliferation of EBV-infected T cells and aggressive and fulminant clinical course often associated with hemophagocytic syndrome, which requires differentiation with medical conditions such as invasive NK cells, leukemia/lymphoma, EBV infection associated lymphoproliferative lesions and primary cutaneous gamma-delta T-cell lymphoma.