Abstract：
BACKGROUND: Advanced-stage mycosis fungoides (MF) and Sézary syndrome (SS) have poor prognosis with median survivals of less than 5 years. Although a variety of treatments are approved for MF/SS patients, durable complete remissions (CR) are rare.
METHODS: Advanced-stage MF or SS patients who achieved CR and maintained in CR or stage IA for more than 10 years were identified by a retrospective search of the principal investigator\'s database.
RESULTS: Of 2266 patients diagnosed with MF or SS, 23 patients with advanced-stage MF/SS (6 IIB, 1 IIIB, 5 IVA1, 3 IVA2, 8 IVB) who achieved CR and maintained in CR or stage IA for ≥ 10 years were identified. As final/curative treatment, 11 patients underwent allogeneic stem cell transplantation (SCT). Most patients presented at young age, underwent SCT with reduced intensity conditioning regimen, had matched related donors, and controllable post-transplant graft versus host disease. Eleven patients were treated with TSEB as part of combined modality protocol in 2 patients and debulking therapy before allogeneic SCT in 9 patients. Five stage IIB patients achieved CR with radiotherapy. Four patients with blood involvement were treated with extracorporeal photopheresis (ECP) in combination with long-term antibiotics and immunomodulatory agents. Long-term antibiotics were given to 14 patients.
CONCLUSIONS: TSEB followed by allogeneic SCT, radiotherapy, ECP plus long-term antibiotics and immunomodulatory agents were the most common curative/final treatments found in our patients. We are reporting the details of our long-term complete responders\' treatment course in the hopes of obtaining more cure responses in the future.
METHODS: Advanced-stage MF or SS patients who achieved CR and maintained in CR or stage IA for more than 10 years were identified by a retrospective search of the principal investigator\'s database.
RESULTS: Of 2266 patients diagnosed with MF or SS, 23 patients with advanced-stage MF/SS (6 IIB, 1 IIIB, 5 IVA1, 3 IVA2, 8 IVB) who achieved CR and maintained in CR or stage IA for ≥ 10 years were identified. As final/curative treatment, 11 patients underwent allogeneic stem cell transplantation (SCT). Most patients presented at young age, underwent SCT with reduced intensity conditioning regimen, had matched related donors, and controllable post-transplant graft versus host disease. Eleven patients were treated with TSEB as part of combined modality protocol in 2 patients and debulking therapy before allogeneic SCT in 9 patients. Five stage IIB patients achieved CR with radiotherapy. Four patients with blood involvement were treated with extracorporeal photopheresis (ECP) in combination with long-term antibiotics and immunomodulatory agents. Long-term antibiotics were given to 14 patients.
CONCLUSIONS: TSEB followed by allogeneic SCT, radiotherapy, ECP plus long-term antibiotics and immunomodulatory agents were the most common curative/final treatments found in our patients. We are reporting the details of our long-term complete responders\' treatment course in the hopes of obtaining more cure responses in the future.
摘要：
背景：晚期真菌病（MF）和Sézary综合征（SS）的预后较差，中位生存期少于5年。尽管MF/SS患者批准了多种治疗方法，持久的完全缓解(CR)很少见。
方法：通过对主要研究者数据库的回顾性搜索，确定了达到CR并维持CR或IA期10年以上的晚期MF或SS患者。
结果：在2266例确诊为MF或SS的患者中，23例晚期MF/SS（6IIB，1IIIB,确定5IVA1，3IVA2，8IVB）达到CR并维持CR或IA期≥10年。作为最终/治愈性治疗，11例患者行同种异体干细胞移植(SCT)。大多数患者在年轻时出现，接受SCT与降低强度预处理方案，有匹配的相关捐赠者，和可控的移植后移植物抗宿主病。11例患者接受了TSEB作为联合模式方案的一部分，其中2例患者接受了TSEB治疗，9例患者接受了同种异体SCT之前的减积治疗。5例IIB期患者放疗获得CR。对4例血液受累患者进行了体外光脱疗法（ECP）联合长期抗生素和免疫调节剂治疗。14例患者接受长期抗生素治疗。
结论：TSEB随后是同种异体SCT，放射治疗,ECP加长期抗生素和免疫调节剂是我们患者中最常见的治愈/最终治疗方法。我们正在报告我们的长期完整应答者的治疗过程的细节，希望在未来获得更多的治疗反应。
方法：通过对主要研究者数据库的回顾性搜索，确定了达到CR并维持CR或IA期10年以上的晚期MF或SS患者。
结果：在2266例确诊为MF或SS的患者中，23例晚期MF/SS（6IIB，1IIIB,确定5IVA1，3IVA2，8IVB）达到CR并维持CR或IA期≥10年。作为最终/治愈性治疗，11例患者行同种异体干细胞移植(SCT)。大多数患者在年轻时出现，接受SCT与降低强度预处理方案，有匹配的相关捐赠者，和可控的移植后移植物抗宿主病。11例患者接受了TSEB作为联合模式方案的一部分，其中2例患者接受了TSEB治疗，9例患者接受了同种异体SCT之前的减积治疗。5例IIB期患者放疗获得CR。对4例血液受累患者进行了体外光脱疗法（ECP）联合长期抗生素和免疫调节剂治疗。14例患者接受长期抗生素治疗。
结论：TSEB随后是同种异体SCT，放射治疗,ECP加长期抗生素和免疫调节剂是我们患者中最常见的治愈/最终治疗方法。我们正在报告我们的长期完整应答者的治疗过程的细节，希望在未来获得更多的治疗反应。
