种痘水疱病样皮肤T细胞淋巴瘤3例及文献复习.pdfVIP

中国皮肤性病学杂志2008年6月第22卷第6期 Chin J Derm Venereol，June．2008，Vo1．22，No．6 ·34l· 种痘水疱病样皮肤T细胞淋巴瘤3例及文献复习 宿斌 ，王宝玺 ，渠涛 ，方凯 ，吴晓燕 ，高洁 ，钟定荣 [摘 要】 目的 归纳、分析种痘水疱病样皮肤T细胞淋巴瘤患者的临床、组织病理特点及治疗和预后，以提高临 床医生对该病的诊治水平。方法 分析3例患者的皮疹特点、伴发症状、辅助检查、组织病理、治疗及预后等临床资料，并 复习相关文献。结果 患者2男1女，发病年龄最早3岁，最晚1O岁。皮损具有多形性，发作时伴高热、淋巴结及肝脾肿 大。组织病理可见真皮至皮下脂肪层内的异型性淋巴样细胞围绕血管浸润，有明显的侵犯血管壁和脂肪小叶现象，伴有 较多的EOS浸润；免疫组化示肿瘤细胞表达CD8，CD45RO，CD43，而不表达CD20，CD30，CD56。皮损组织基因重排检查 TCR一 (+)。Epstein—Barr病毒原位杂交(+)。外周血EOS明显升高，LDH酶稍高，EB病毒抗体(+)。例1行化疗， 后死亡；例2给予强的松17服，例3接受防蚊子叮咬及抗病毒治疗，均长期缓解。结论 种疽水疱病样皮肤T细胞淋巴瘤 是一种新的恶性多系统疾病，发病可能与EBV感染以及蚊子叮咬有关。不同的患者恶性程度不一，治疗及预后亦不同。 [关键词] 淋巴瘤；种痘水疱病样；T细胞；Epstein—Barr病毒 [中图分类号】 R 739．5 [文献标识码】 A [文章编号】 1001—7089(2008)06—0341—04 Hydroa Vacciniforme—like Cutaneous T cell Lymphoma：Case Reports and Literature Review SU Bin，WANG Bao—xi，Qu Tao，et al (Department of dermatology，Peking Union Medical College Hospital，Beijing 100730，China) Abstract：Objective The purpose of this study was to describe 3 cases of hydroa vacciniforme—like T cell lymphoma，to discuss its clinicaI．histopathologic，and immunohistochemicaI features．Methods 3 patients with the disorder from PUMC hos— pital were enrolled in the study．The clinical and histiopathoIogical features were analyzed．Results ALl the patients were chil- dren，1 female and 2 were male．Skin lesions presented with polymorphous lesions accompanied with high fever and hepatospleno— megaly．Histopathological findings revealed that diffuse and dense infiltrate of lymphoid cells with anasplastic nuclei in the middle and IOW potion of the dermis as welI as the SUbcutis：Noeplastic cells invaded blood vessels and subcutaneous fat．with an obviously angiocentric pattern．Moderate numbers of eosinophils were a1so present in the dermal infi ltrates．The heteromorphic cells were