Extrakorporale Photopherese: Studie zum Langzeiteffekt bei chronischer Graft-versus-Host-Reaktion

P. Lehmann
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Abstract

Background: Extracorporeal photopheresis (ECP) is a second-line therapy for steroid-refractory chronic graft-versus-host disease (cGVHD). Objective: 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. Patients and Methods: 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. Results: 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. Discussion and Conclusion: ECP was an effective long-term therapy for oral and cutaneous cGVHD: consequently, dose levels of therapeutic immunosuppression could be reduced.
超自然光子:研究对慢性病与背心反应的长期影响
背景:体外光移植术(Extracorporeal photopheresis, ECP)是治疗类固醇难治性慢性移植物抗宿主病(cGVHD)的二线疗法。目的:我们根据cGVHD的皮肤表型描述ECP的长期疗效和耐受性,并报告在这种情况下对免疫抑制药物的需求减少。患者和方法:纳入了2010年10月至2016年5月在单一中心内接受ECP治疗的14例皮肤和/或粘膜cGVHD患者(8名女性)。最终分析包括接受ECP治疗至少12个月的患者。我们使用病变特异性临床评分和记录全身免疫抑制剂剂量的变化来前瞻性评估ECP的疗效。结果:14例患者中有10例(71%)出现皮肤硬化。从第9个月开始,所有患者的mRODNAN评分都下降了,在12个月和36个月分别下降了40%和77%。6名患者(43%)出现皮肤苔藓样病变:到第3个月,所有患者的评分都降低了,到第12个月,评分降低了93%。5名患者(36%)出现口腔粘膜苔藓样病变:这些评分在12个月时下降了55%,在33个月时下降了100%。所有患者全身免疫抑制剂的使用均有所减少;4例患者2年后全部停用免疫抑制药物。3例患者完全缓解后停用ECP。未观察到与ecp相关的主要不良反应。讨论与结论:ECP是口服和皮肤cGVHD的有效长期治疗方法,可降低治疗性免疫抑制的剂量水平。
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