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[目的]分析不同期蕈样肉芽肿(MF)/Sézary综合征(SS)的临床特征和治疗方法,加深对其认识。[方法]总结华山医院皮肤科1985年1月至2005年12月诊治的236例MF/SS患者的临床资料以及随访情况。[结果]236例中MF226例,男女比例为1.9∶1,诊断时年龄10~91岁,病程平均81.9个月。SS10例,男性8例,女性2例,诊断时年龄34~74岁。早期MF治疗有效率为单用光(化学)疗法93%,单用免疫调节剂92%,两者合用89%。随访至今的110例中,39例无病生存,48例带病生存,4例死于其他原因,19例死于本病。[结论]MF可发生于各年龄段,男女比例接近2∶1,病程较长。早期MF主要行光(化学)治疗和免疫调节剂治疗,单独或联合应用有效率均较高。晚期MF和SS易诊断,但预后欠佳,治疗以系统性化疗辅以免疫调节剂为主。
[Objective] To analyze the clinical features and treatment methods of different stages of mycosis fungoides (MF) / Sézary syndrome (SS) and to deepen their understanding. [Methods] The clinical data and follow-up of 236 patients with MF / SS who were treated in Department of Dermatology, Huashan Hospital from January 1985 to December 2005 were summarized. [Results] There were 236 cases of MF226 cases, the ratio of male to female was 1.9: 1, the age of diagnosis was from 10 to 91 years old, and the course of disease was 81.9 months on average. SS10 cases, 8 males and 2 females, the age of diagnosis of 34 to 74 years old. Early MF treatment efficiency of light alone (chemotherapy) 93%, single use of 92% immunomodulator, the two combined 89%. Of the 110 patients who were followed up, 39 were disease-free, 48 were alive, 4 died of other causes, and 19 died of this disease. [Conclusion] MF can occur in all age groups, the ratio of male to female is close to 2: 1 and the course is longer. Early MF mainly light (chemical) treatment and immunomodulator treatment, alone or in combination with high efficiency. Late MF and SS are easy to diagnose, but the prognosis is not good, the treatment of systemic chemotherapy supplemented by immunomodulators.