牛痘样水疱病样皮肤T细胞淋巴瘤

Hidroa vacciniforme-like cutaneous T cell lymphoma of three cases

目的:根据3例种痘样皮肤T细胞淋巴瘤(hydroa vacciniforme-likeCTCL)患者的临床表现、治疗及转归,进一步探讨该病的诊断和治疗。方法:分析3例hydroa vacciniforme-like CTCL患者的临床资料、实验室检查、治疗及转归。结果:3例患者均为幼年发病,皮损开始出现在曝光部位,反复发作,数月或数年后进展性或逐渐蔓延至非曝光部位,且伴有发热等全身症状。皮损组织病理显示真皮内致密的淋巴样细胞浸润达真皮下层甚至脂肪层,常侵犯血管;免疫组化组织病理显示浸润细胞以CD8(+)细胞为主;T细胞受体γ基因(TCRγ基因)呈单克隆性重排;EB(Epstein-Barr)病毒原位杂交(+)。结论:该病与EB病毒感染有关。该病预后差,但干扰素治疗可改善症状。

Objective： To study the diagnosis standard, therapy and prognosis in 3 cases of hydroa vacciniforme-like cutaneous T cell lymphoma （HV-like CTCL）. Methods： The clinical data, laboratory examinations of 3 cases of HV-like CTCL were analyzed. Results： All patients suffered from the condition in childhood, Their impressive cutaneous rash characterized by edema, blisters, ulcers, crusts, and scars, resembling hydroa vacciniforme, were seen mainly in light-exposed and non-exposed areas. They had fever, and one of the three patients was often found to have high-grade fever, liver damage. The pathology of skin showed that it consisted of dense lymphomatous T cell infiltration of the epidermis, dermis and subeutis with variable exocytosis and angiocentricity, immunohistochemieal staining showed that most of the infiltrating large lymphoid cells were positive for CD45-RO and CD8, and the TCR-γ gene rearrangement study revealed a monoclonal band presented in the DNA extracted from the specimens. In situ hybridization demonstrated a latent infection of Epstein-Barr virus in the majority of lymphoid cells in the dermis. Conclusion： HV-like CTCL may be remitted in the treatment with interferon（IF）.