儿童种痘样水疱病样皮肤T细胞淋巴瘤7例分析

Analysis on Hydroa Vacciniforme-like Cutaneous T-cell Lymphoma in Seven Children

[目的]探讨种痘样水疱病样皮肤T细胞淋巴瘤患儿的临床表现、病理特征、免疫组化及治疗和预后。[方法]分析7例患儿的皮疹特点、伴发症状、实验室检查、组织病理、免疫组化,治疗及预后等资料。[结果]患儿皮损具有多形性,包括肿胀、红斑、丘疹、水疱、结节、溃疡、瘢痕,发作时伴高热、淋巴结、肝或(和)脾肿大。组织病理检查可见真皮内较致密的异型淋巴细胞,围绕血管及附属器浸润,可深达脂肪层;免疫组化示肿瘤细胞表达CD3、CD45RO、TIA-1、Granzyme-B、CD4或CD8。6例行Epstein-Barr(EB)病毒原位杂交均阳性。除例6患儿失访外,其余6例患儿均采用小剂量强的松、阿昔洛韦、干扰素α2b、异维A酸、反应停等药物治疗,治疗期间病情均得到缓解,随访至今无患儿死亡。[结论]种痘样水疱病样皮肤T细胞淋巴瘤是一种罕见肿瘤,发病与EB病毒慢性活动性感染相关,不同患者恶性程度不一,治疗及预后亦不同。

[Purpose] To investigate the clinical presentation,histopathology,immunohistochemistry,treatment and prognosis of Epstein-Barr virus（EBV）-associated hydroa vacciniforme-like cutaneous T-cell lymphoma. [Methods] The clinical,morphologic,and immunophenotypical features,treatment and prognosis of 7 cases in children were analyzed.[Results] All the patients had skin lesions,including edema,papulovesicles,blistering,ulceration,and scarring,and they also had systemic manifestations,such as intermittent fever,hepatosplenomegaly or lymph node enlargement. Histopathologic examination showed an atypical lymphocytic infiltrate with angiotropism and angiocentricity,and dense lymphoid infiltration throughout the dermis and subcutaneous tissue. The atypical lymphocytes were positive for CD3,CD45 RO,TIA-1,Granzyme-B,CD8 or CD4. Tumor cells positive for EBV encoded RNA（EBER） were detected in cutaneous infiltrates in 6 cases. Six patients were treated with low dosage of prednisone,acyclovir,interferon α2b,isotretinion,thalidomide,and achieved complete remission. Patient No. 6 was lost,the rest of six patients were alive until now. [Conclusion] Hydroa vacciniforme-like cutaneous T-cell lymphoma is a rare lymphoma occurring mainly in children. Chronic active EBV infection（CAEBV） has been associated with this disease. Different patients have different courses and different treatments,which lead to different prognosis.