淋巴血浆置换疗法治疗难治性重症免疫相关性皮肤病疗效观察  

Efficacy of combined lymphoplasma exchange for the treatment of severe refractory immunerelated skin diseases:a clinical observation

作　　者：饶诗佳 李碧娟[2] 李吉[3] 谢红付[3] 陈明亮[3] 赵爽[3] 王亚玲 施为[3] 陆前进[1] Rao Shijia;Li Bijuan;Li Ji;Xie Hongfu;Chen Mingliang;Zhao Shuang;Wang Yaling;Shi Wei;Lu Qianjin(Department of Dermatology,The Second Xiangya Hospital of Central South University,Changsha 410011,China;Department of Blood Transfusion,Xiangya Hospital,Central South University,Changsha 410008,China;Department of Dermatology,Xiangya Hospital,Central South University,Changsha 410008,China)

机构地区：[1]中南大学湘雅二医院皮肤科,长沙410011 [2]中南大学湘雅医院输血科,长沙410008 [3]中南大学湘雅医院皮肤科,长沙410008

出　　处：《中华皮肤科杂志》2019年第1期16-19,共4页Chinese Journal of Dermatology

摘　　要：目的观察淋巴血浆置换治疗难治性重症免疫相关性皮肤疾病的疗效。方法对2013年5月至2015年10月于中南大学湘雅医院皮肤科收治的8例常规治疗无效或伴有多脏器功能受损的中毒性表皮坏死松解症、药物超敏反应综合征、寻常型天疱疮、类天疱疮、副肿瘤性天疱疮患者,采用淋巴血浆置换治疗,评估疗效并观察不良反应。结果6例患者淋巴血浆置换治疗1次后症状即显著改善,最终痊愈。1例寻常型天疱疮患者对大剂量糖皮质激素和免疫抑制剂治疗抵抗,淋巴血浆置换治疗期间皮疹一度缓解,但治疗结束后皮疹反复。1例大疱性类天疱疮患者在淋巴血浆置换治疗次日出现暴发性水疱,考虑为异体血浆过敏反应。血浆置换前后8例患者外周血白细胞、淋巴细胞、中性粒细胞、血小板计数等实验室指标均无明显变化。随访3~5年,除1例大疱性类天疱疮患者1年后死于播散性结核感染,其他患者均已康复,无复发。结论淋巴血浆置换疗法对重症免疫相关性皮肤病有良好疗效,需关注潜在的输血反应和过敏反应。Objective To evaluate the clinical efficacy of lymphoplasma exchange (LPE) for the treatment of severe refractory immune-related skin diseases.Methods From May 2013 to October 2015, 8 patients with toxic epidermal necrolysis, drug-induced hypersensitivity syndrome (DIHS) , pemphigus vulgaris, pemphigoid or paraneoplastic pemphigus were enrolled from Department of Dermatology, Xiangya Hospital, Central South University, who showed no response to conventional therapy or presented with multiple organ dysfunction. After the treatment with LPE, the efficacy was evaluated, and adverse reactions were observed. Results After one session of LPE therapy, 6 patients received marked improvement, and were cured at last. In 1 patient with pemphigus vulgaris who was resistant to the treatment with high doses of glucocorticoids and immunosuppressive agents, the rashes regressed during the treatment with LPE, but recurred after the end of treatment. One patient with bullous pemphigoid presented with eruptive blisters on the next day after the treatment with LPE, which were considered as allergic reactions to allogeneic plasma. There were no obvious differences in white blood cell count, lymphocyte count, neutrophil count and blood platelet count in the peripheral blood of 8 patients before and after the treatment with LPE. During the follow-up of 3-5 years, all of the patients were recovered without recurrence, except 1 patient with bullous pemphigoid who died of disseminated tuberculosis after 1 year.Conclusion LPE is effective for the treatment of severe immune-related skin diseases, but attention should be paid to potential transfusion reaction and allergic reactions.

关 键 词：血浆置换 表皮坏死松解症 中毒性 天疱疮 类天疱疮 大疱性 药物超敏反应综合征 副肿瘤性天疱疮 

分 类 号：R751.05[医药卫生—皮肤病学与性病学]