皮下脂膜炎性T细胞淋巴瘤19例临床分析及相关文献复习

Clinical analysis of 19 cases of subcutaneous panniculitis T-cell lymphoma with literature review

背景与目的:皮下脂膜炎性T细胞淋巴瘤(subcutaneous panniculitis Tcell lymphoma,SPTCL)是一种非常罕见的原发皮肤淋巴瘤。本文旨在分析SPTCL的临床特点、治疗与预后,增加对该病的认识。方法:回顾性分析中山大学肿瘤防治中心和中山大学附属第一医院2001年1月至2007年7月收治的19例SPTCL患者的临床特征、治疗经过和随访结果。结果:全组患者中位年龄为36岁,7例患者出现皮肤以外的结外侵犯,10例患者治疗前乳酸脱氢酶(LDH)异常升高,1例患者伴噬血细胞综合征。患者接受以化疗为主的治疗,化疗方案包括CHOP、m-ATT等。中位随访时间56个月。Kaplan-Meier分析全组患者中位生存时间为40个月,2年预期总生存为56%。8例持续完全缓解17～70个月,均采用强烈化疗方案;其中6例在化疗后接受了放疗。单因素分析(log-rank检验)提示,性别、B症状、皮肤以外的结外侵犯、治疗前血常规和LDH水平对预后有影响。结论:SPTCL可通过化疗与放疗相结合的综合治疗治愈,把有效且无交叉耐药的化疗方案交替应用或使用有效的强烈化疗,继以全身电子线照射,有可能进一步提高缓解率和总生存率。

Background and Objective： Subcutaneous panniculitis T-cell lymphoma （SPTCL） is a rare subtype of primary cutaneous lymphoma. This study was to analyze the clinical characteristics, treatment and prognosis of SPTCL. Methods： Clinical data of 19 SPTCL patients, treated at Cancer Center and the First Affiliated Hospital of Sun Yat-sen University from January 2001 to July 2007, were analyzed. Results： The median age of the patients was 36 years. Seven patients had skin-excluded extra-nodal involvement; ten had lactate dehydragenase （LDH） elevation before treatment; one had hemophagocytic syndrome. Most of the parents received chemotherapy, including CHOP regimen, modified-alternative triple therapy （m-ATT）, and Hyper-CVAD/HD-MA regimen. The median follow-up was 56 months, The median survival was 40 months, and the 2-year expected overall survival rate was 56%. Eight patients who received treatment of intensive chemotherapy had continous remission of 17-70 months; six of them underwent radiotherapy after chemotherapy. Univariate analysis （log-rank test） showed that sex, B symptoms, skin-excluded extra-nodal involvement, and pre- treatment blood cell count and LDH level affected the prognosis. Conclusions： SPTCL might be cured by high dose chemotherapy combined with whole body irradiation. The regimens which are effective without crossing resistance or more intensive may improve the response rate and overall survival.