Dermatology Department, University Hospital and Paul Sabatier University of Toulouse, Toulouse, France.
Hematology Department, University Institute of Cancer-Oncopole, Toulouse, France.
Dermatology. 2018;234(1-2):23-30. doi: 10.1159/000488238. Epub 2018 May 22.
Extracorporeal photopheresis (ECP) is a second-line therapy for steroid-refractory chronic graft-versus-host disease (cGVHD).
We describe the long-term efficacy and tolerability of ECP according to the cutaneous phenotype of cGVHD and report on the reduced need for immunosuppressant drugs in this setting.
Fourteen patients (8 females) with cutaneous and/or mucosal cGVHD, treated with ECP between October 2010 and May 2016 within a single center, were included. Final analyses included patients who had received ECP for at least 12 months. We prospectively evaluated the efficacy of ECP using lesion-specific clinical scores and by recording changed doses of systemic immunosuppressants.
Of the 14 patients, sclerotic skin lesions were present in 10 (71%). The mRODNAN score decreased in all patients from month 9 onwards, with 40 and 77% reductions at 12 and 36 months, respectively. Six patients (43%) presented with cutaneous lichenoid lesions: this score was reduced in all patients by month 3, reaching a 93% reduction by month 12. Five patients (36%) experienced oral mucosal lichenoid lesions: these scores were decreased by 55% at month 12 and by 100% by month 33. The use of systemic immunosuppressants was reduced in all patients; 4 patients could stop all immunosuppressant drugs after 2 years. ECP was stopped in 3 patients after a complete response. No major ECP-associated adverse effects were observed.
ECP was an effective long-term therapy for oral and cutaneous cGVHD: consequently, dose levels of therapeutic immunosuppression could be reduced.
体外光分离术(ECP)是治疗类固醇难治性慢性移植物抗宿主病(cGVHD)的二线治疗方法。
我们根据 cGVHD 的皮肤表型描述 ECP 的长期疗效和耐受性,并报告在此情况下免疫抑制剂药物需求减少。
2010 年 10 月至 2016 年 5 月,在单一中心接受 ECP 治疗的 14 例(8 名女性)皮肤和/或黏膜 cGVHD 患者纳入本研究。最终分析包括接受 ECP 治疗至少 12 个月的患者。我们前瞻性地使用特定于病变的临床评分和记录全身免疫抑制剂剂量变化来评估 ECP 的疗效。
14 例患者中,10 例(71%)存在硬化性皮肤病变。从第 9 个月开始,mRODNAN 评分在所有患者中均降低,分别在 12 个月和 36 个月时降低了 40%和 77%。6 例(43%)患者表现为皮肤苔藓样病变:所有患者在第 3 个月时该评分均降低,在第 12 个月时降低了 93%。5 例(36%)患者出现口腔黏膜苔藓样病变:在第 12 个月时评分降低了 55%,在第 33 个月时降低了 100%。所有患者的全身免疫抑制剂使用量减少;4 例患者在 2 年后可以停止所有免疫抑制剂药物。3 例患者在完全缓解后停止 ECP。未观察到与 ECP 相关的严重不良反应。
ECP 是治疗口腔和皮肤 cGVHD 的有效长期治疗方法:因此,可以减少治疗性免疫抑制药物的剂量。