EBV阳性T细胞淋巴组织增殖性疾病3例临床病理观察

Epstein-Barr virus-positive of T-cell lymphoproliferative disorders: a clinicopathologic analysis of 3 cases

目的探讨EBV阳性T细胞淋巴组织增殖性疾病的临床病理特征。方法报道3例EBV阳性T细胞淋巴组织增殖性疾病，从临床表现、病理形态学、免疫表型、EBER原位杂交和T细胞受体基因重排等方面进行研究，并结合相关文献进行讨论。结果1例成人型系统性EBV阳性T细胞淋巴组织增殖性疾病以发热起病，肝脾受累明显，肝窦和脾窦内小淋巴细胞浸润伴较多嗜血细胞，免疫组化示浸润淋巴细胞以T细胞为主，EBER（＋），基因重排未见单克隆条带。2例种痘水疱病样淋巴瘤以面部、躯干及四肢反复出疹样皮损伴破溃、痂皮形成为主要表现，真皮及皮下脂肪组织内见小～中等大小淋巴细胞浸润，以血管中心性浸润为特征，表达T细胞抗原和EBER，2例均显示TCR受体基因单克隆性重排。结论EBV阳性T细胞淋巴组织增殖性疾病临床表现不特异，组织学易误诊为炎性反应性病变或嗜血细胞综合症，T细胞受体基因重排能够帮助明确本类疾病的恶性本质。

Objective To investigate the clinicopathological characters of Epstein-Barr virus-positive of T cell lymphoproliferative disorders （ EBV ＋ T-cell LPDs）. Methods We analysed the clinical and pathological datas of 3 EBV ＋ T-cell LPDs. Immunohistochemistry, in situ hybridization for EBV-encoded RNA （EBER） and polymerase chain reaction （PCR） analysis based on BIOMED-2 system for T-cell receptor （TCR） gene rearrangements were performed on paraffin-embedded tissues from all 3 patients. The follow-up data were collected. Results Three patients included 1 systemic EBV-positive T-cell lymphoproliferative disorders （ SEBV ＋ T-cell LPDs） in elderly man and 2 adolescent girls who suffered from hydroa vaceiniforme-like lymphoma. All three patients had EBER ＋ and CD3 ＋ T-cells in their biopsy tissues. Molecular analysis revealed monoclonal T-cells in 2 cutaneous cases, but not found in SEBV ＋ T-cell LPDs case. Conclusion EBV＋ T-cell LPDs are life-threaten diseases, elonality analysis of infiltrating T-cells may help confirming the diagnosis.