Richardson Stephen K, Lin Julie H, Vittorio Carmela C, Kim Ellen J, Yoon Jessica S, Junkins-Hopkins Jacqueline, Rook Alain H
Department of Dermatology, University of Pennsylvania, Philadelphia, USA.
Clin Lymphoma Myeloma. 2006 Nov;7(3):226-32. doi: 10.3816/CLM.2006.n.063.
The goal of this study was to evaluate the clinical response rate of patients with Sézary syndrome (SS) to multimodality immunomodulatory therapy consisting of extracorporeal photopheresis in combination with >/= 2 systemic biologic response modifiers (interferon-, interferon-, retinoids, and/or sargramostim) and psoralen plus UV-A.
Twenty-eight patients who met established criteria for SS were treated with multimodality immunomodulatory therapy at the Hospital of the University of Pennsylvania between January 2000 and December 2002. All patients received > 6 cycles of extracorporeal photopheresis. Patients were categorized into groups based on their response to therapy.
An overall clinical response of 89% was achieved with multimodality immunomodulatory therapy. Twenty-nine percent of patients exhibited a complete response, characterized by no evidence of cutaneous disease and a Sézary count 5%. Sixty-one percent exhibited a partial response. Eleven percent were nonresponders.
Based on our experience, multimodality immunomodulatory therapy is an exceptionally effective treatment for SS. The durability of response and impact on overall survival remains to be determined; however, this approach offers an appealing alternative to treatments associated with higher morbidity rates.
本研究的目的是评估 Sézary 综合征（SS）患者对多模式免疫调节治疗的临床反应率，该治疗包括体外光化学疗法联合≥2 种全身性生物反应调节剂（干扰素、维甲酸和/或沙格司亭）以及补骨脂素加紫外线 A。
2000 年 1 月至 2002 年 12 月期间，28 例符合 SS 既定标准的患者在宾夕法尼亚大学医院接受了多模式免疫调节治疗。所有患者均接受了超过 6 个周期的体外光化学疗法。根据患者对治疗的反应进行分组。
多模式免疫调节治疗实现了 89%的总体临床反应率。29%的患者表现出完全缓解，其特征为无皮肤疾病证据且 Sézary 细胞计数<5%。61%的患者表现出部分缓解。11%的患者无反应。
根据我们的经验，多模式免疫调节治疗是 SS 的一种极其有效的治疗方法。反应的持久性及其对总生存期的影响仍有待确定；然而，这种方法为与较高发病率相关的治疗提供了一种有吸引力的替代方案。
