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[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。
[Objective] To investigate the clinical manifestations, pathological features, immunohistochemistry, treatment and prognosis of children with acne-like blister-like cutaneous T-cell lymphoma. [Method] The rash characteristics, concomitant symptoms, laboratory examination, histopathology, immunohistochemistry, treatment and prognosis of 7 children were analyzed. [Results] Lesions in children were pleomorphic, including swelling, erythema, papules, blisters, nodules, ulcers, scars, seizures with hyperthermia, lymph nodes, liver or (and) splenomegaly. Histopathological examination showed dense dendritic lymphocytes in the dermis, which infiltrated into blood vessels and adnexal vessels and reached the fat layer. Immunohistochemistry showed that the tumor cells expressed CD3, CD45RO, TIA-1, Granzyme-B, CD4 or CD8.6 Routine Epstein-Barr (EB) virus in situ hybridization was positive. In addition to the cases of 6 children lost, the remaining 6 cases were treated with low-dose prednisone, acyclovir, interferon α2b, isotretinoin, reaction stop and other drugs, the disease was alleviated during treatment, follow-up No child has died so far. [Conclusion] Pox-like blister-like cutaneous T-cell lymphoma is a rare tumor associated with chronic active infection of Epstein-Barr virus. Different patients have different degrees of malignancy and different treatment and prognosis.