Department of Dermatology, Venereology and Allergology, University Medical Centre Mannheim, Ruprecht-Karls-University of Heidelberg, Mannheim, Germany.
J Dtsch Dermatol Ges. 2010 Jun;8(6):428-38. doi: 10.1111/j.1610-0387.2010.07319.x. Epub 2010 Feb 18.
Extracorporeal photopheresis (ECP) is recommended for the treatment of Sézary syndrome (SS), the leukemic variant of cutaneous T-cell lymphoma (CTCL). Several combination therapies are used to increase response rates to ECP.
We report our experience with the combination therapy of ECP, interferon-alpha, PUVA and topical corticosteroids in SS.
The treatment outcome in 12 SS patients was retrospectively analyzed and showed an overall response rate to this combination treatment of 42 % with 4/12 patients achieving a partial remission and 1/12 patients a stable disease. The median overall survival time was 42 months. We investigated several clinical and laboratory parameters as an indicator for a response to treatment in our patient cohort. A combined analysis of the erythroderma assessment scale, WBC, LDH, CD4/CD8 ratio and the number of Sézary cells revealed that a reduction of several parameters significantly correlated with response to treatment. The parameters which correlated best with response were number of Sézary cells, CD4/CD8 ratio and WBC.
The investigated combination therapy was effective and well-tolerated in a subgroup of SS patients but needs to be evaluated in a larger patient population.
体外光分离（ECP）被推荐用于治疗蕈样肉芽肿（SS），即皮肤 T 细胞淋巴瘤（CTCL）的白血病变体。几种联合疗法用于提高 ECP 的反应率。
我们报告了 ECP、干扰素-α、PUVA 和局部皮质类固醇联合治疗 SS 的经验。
对 12 例 SS 患者的治疗结果进行了回顾性分析，显示该联合治疗的总体反应率为 42%，其中 4/12 例患者部分缓解，1/12 例患者病情稳定。中位总生存时间为 42 个月。我们研究了几个临床和实验室参数作为我们患者队列中治疗反应的指标。红细胞评估量表、白细胞计数（WBC）、乳酸脱氢酶（LDH）、CD4/CD8 比值和蕈样肉芽肿细胞数量的综合分析表明，几个参数的减少与治疗反应显著相关。与反应相关性最好的参数是蕈样肉芽肿细胞数量、CD4/CD8 比值和 WBC。
在 SS 患者的亚组中，所研究的联合治疗是有效且耐受良好的，但需要在更大的患者群体中进行评估。
