PDF(Pubmed)
Abstract：
Mycosis fungoides and Sézary syndrome are rare and largely incurable types of cutaneous T-cell lymphoma with limited therapeutic options. In 1984 Bunn et al. reported that interferon alpha is an efficient monotherapy in cutaneous T-cell lymphoma and 14 years later it was shown in a prospective, randomized trial that a combination of interferon alpha and psoralen plus ultraviolet A therapy (PUVA) is most efficient in the treatment of cutaneous T-cell lymphoma. Since then interferon alpha as single agent or, most often, in combination with phototherapy and/or retinoids has been integrated as standard of care in cutaneous T-cell lymphoma guidelines worldwide. However, production of interferon alpha was discontinued recently worldwide and pegylated interferon alpha-2a (PEG-IFNα) has been used as an alternative therapy. In contrast to numerous interferon alpha studies, only a few studies focusing on PEG-IFNα are available. Therefore, the aim of this study was to conduct a retrospective data collection to report on the efficacy, adverse events and therapy regimens of PEG-IFNα in cutaneous T-cell lymphoma. In 28 patients with cutaneous T-cell lymphoma treated in Germany and in the Netherlands, 36% of patients achieved complete remission, 36% partial remission and 29% stable disease. Eighteen percent of patients developed adverse events during therapy, which led to the discontinuation of PEG-IFNα therapy in 2 patients. The most common concomittant therapies were oral PUVA phototherapy and local radiotherapy. In conclusion, PEG-IFNα, especially in combination with skin-directed therapies, is an effective treatment option for cutaneous T-cell lymphoma in clinical practice.
摘要：
真菌肉芽肿和Sézary综合征是皮肤T细胞淋巴瘤的罕见且大部分无法治愈的类型，治疗选择有限。1984年Bunn等人。报道，干扰素α是一种有效的单一疗法在皮肤T细胞淋巴瘤和14年后，随机试验表明，干扰素α和补骨脂素加紫外线A疗法（PUVA）的组合在皮肤T细胞淋巴瘤的治疗中最有效。从那时起，干扰素α作为单一药剂或，大多数情况下,与光疗和/或类维生素A的组合已被纳入全球皮肤T细胞淋巴瘤指南中作为护理标准。然而,干扰素α的生产最近在世界范围内停止，聚乙二醇化干扰素α-2a（PEG-IFNα）已被用作替代疗法。与许多干扰素α研究相反，只有少数研究关注PEG-IFNα。因此,这项研究的目的是进行回顾性数据收集，以报告疗效，PEG-IFNα在皮肤T细胞淋巴瘤中的不良事件和治疗方案。在德国和荷兰接受治疗的28例皮肤T细胞淋巴瘤患者中，36%的患者达到完全缓解,36%部分缓解，29%病情稳定。18%的患者在治疗期间出现不良事件，导致2例患者停止PEG-IFNα治疗。最常见的伴随疗法是口服PUVA光疗和局部放疗。总之,PEG-IFNα，特别是与皮肤定向疗法相结合，是临床上皮肤T细胞淋巴瘤的有效治疗选择。
