儿童系统性EB病毒阳性T细胞淋巴增殖性疾病临床病理学分析

目的：探讨儿童系统性EB病毒（Epstein-Barr virus， EBV）阳性T细胞淋巴增殖性疾病（EBV-positive T-cell lymphoproliferative disease of childhood，儿童EBV+ TLPD）的临床病理学特征。方法：对3例儿童EBV+ TLPD进行临床特点、病理学形态及免疫表型特征比较，EBV原位杂交和T细胞受体γ（T cell receptor γ，TCRγ）基因重排检测。结果：3例儿童EBV+ TLPD患儿发病年龄分别为2岁、7岁和10岁。就诊时均表现为发热，肝、脾、淋巴结肿大，肝功能异常，例2患儿伴有皮疹症状。实验室检查证实体内存在EBV感染。病理组织形态：淋巴结结构破坏，扩张副皮质区内毛细血管后微静脉树枝状增生，伴多量小至中等大异型淋巴细胞弥漫增生。免疫组织化学：肿瘤细胞表达细胞毒T细胞相关标记：CD3、CD5、T-bet和TIA-1均阳性，粒酶B 2例阳性，CD4和CD8 2例双阳性、1例双阴性，CD56、CD21和CXCL13均阴性；原位杂交检测3例均EBV阳性；TCRγ基因PCR检测2例阳性。结论：儿童EBV+ TLPD是一种少见的活化细胞毒T细胞的外周T细胞淋巴瘤，EBV原位杂交和分子克隆技术分析有助于诊断。我国病例与国际报道病例临床病理特征基本一致。

Clinical and pathological analysis of three cases of childhood systemic EB virus positive T-cell lymphoproliferative disease

Objective:To explore the clinicopathologic features of systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood(EBV+TLPD).Methods: Three cases of EBV+ TLPD of childhood were studied by analyzing the clinical features,morphology,immunophenotypings,EBER-1 in situ hybridization(ISH)and T-cell receptor(TCR)γ gene rearrangement.Results: The age of onset of the three patients was 2,7 and 10 years,respectively.They all presented with fever,hepatosplenomegaly and liver failure,accompanied by lymphadenopathy.Skin rash occurred in case 2.Laboratory tests confirmed EBV infection.Lymph node biopsy showed effacement of normal architecture.The residue T zone was significantly expanded,with marked proliferation of arborizing high endothelial venules and prominent infiltration of small to medium-sized atypical lymphocytes.The immunostaining showed cytotoxic T cell phenotypes,e.g.CD3,CD5,T-bet and TIA-1 were all positive,while granzyme B were positive in two cases;CD4 and CD8 were double positive in two cases,but double negative in one case;CD56,CD21and CXCL13 were negative in all the cases.EBER-1 could be detected in all the cases by ISH.Monoclonal rearrangement of TCRγ gene was detected in two cases by polymerase chain reaction(PCR).Conclusion: EBV+ TLPD of childhood is a rare type of peripheral T cell lymphoma.EBV detection by ISH and molecular cloning technique analysis could contribute to the diagnosis.The clinicopathologic characteristics of our cases are consistent with that reported in international literature.