蕈样肉芽肿和Sezary综合征:113例患者的治疗方法和结果

作     者：Anadolu R.Y. Birol A. Sanli H. 焦婷 

作者机构：Dr. Koza Sok Yamevler 114/86 06670 Ankara Turkey 

出 版 物：《世界核心医学期刊文摘（皮肤病学分册）》 (Digest of the World Core Medical JOurnals:Dermatology)

年 卷 期：2005年第1卷第12期

页      面：60-60页

学科分类：1002[医学-临床医学] 100214[医学-肿瘤学] 10[医学] 

主　　题：蕈样肉芽肿 Sezary 疾病晚期 喷司他丁 完全缓解率 晚期病例 依从性 补骨脂素 统计学特征 氟达拉滨 

摘      要：Background: Mycosis fungoides (MF) and Sezary syndrome (SS) are the most common forms of cutaneous T- cell lymphoma (CTCL). Various topical and systemic therapeutic alternatives are available, but there is no standard or definite curative treatment regimen. When making a decision about the appropriate treatment modality, the age and compliance of the patient, stage of the disease, treatment accessibility, and previous treatment history should be considered. Aim: To determine the therapeutic response of patients with MF and SS to different treatment modalities. Patients were evaluated with respect to their clinical and demographic features. Methods: One hundred and thirteen patients diagnosed clinically and dermatopathologically with MF and SS between March 1984 and June 2001 were included in the study. Results: Of the 113 patients studied, 110 had a diagnosis of MF and three had a diagnosis of SS; 101 patients (89.4% ) were diagnosed with early stage (IA, IB, IIA) and 12 (10.6% ) with late stage (IIB, III, IVA, IVB) disease. The age at diagnosis varied between 12 and 81 years (mean, 45.6 ± 15.8 years). Fifty- five (48.7% ) patients were male and 58 (51.3% ) were female. The duration of the skin lesions varied between 1.5 months and 32 years (mean, 6.1 years). Psoralen plus UVA (PUVA)was the most commonly used initial treatment modality in early stage disease (91% ), with a complete remission (CR)rate of 80.4% . With PUVA + interferon- α (INF- α ) treatment, CR was 57% in the early stages and 33.3% in the late stages. For late stage disease, systemic therapies, such as pentostatin, gemcitabine, and fludarabine, alone or in combination with INF- α , were preferred. Of the 113 patients, eight (7% of the total and 57.1% of the advanced stage cases) died of MF; 21.4% of the late stage patients showed partial remission and 14.2% showed CR. None of the patients diagnosed with early stage disease died of MF, but two (1.9% ) progressed to late stage disease. Conclusions: PUV