Abstract：
Background: Mycosis fungoides (MF) and Sezary Syndrome (SS) comprise over half of all Cutaneous T-cell lymphoma diagnoses. Current risk stratification is largely based on TNMB staging, few research investigated the prognostic value of clinical exams. Current systemic therapy for advanced disease includes immunomodulatory drugs, chemotherapy, and HADC inhibitors. Few clinical trials or retrospective research compared the efficacy of different drugs.Method: Here, we performed a retrospective analysis of prognostic factors and treatment outcomes of 92 patients diagnosed with MF/SS at the Peking Union Medical College Hospital from 2013-2023.Results: Cox regression analysis identified that age ≥ 50 years, WBC ≥ 8 × 109/L, serum LDH ≥ 250U/L, β2-MG ≥ 4.50 mg/L, and stage IV were associated with reduced overall survival, age ≥ 50 years, serum LDH ≥ 250U/L and stage IV were associated with reduced progression free survival. Kaplan-Meier analysis established that immunomodulatory therapy was associated with longer progression free survival.Conclusion: These results suggested new factors in predicting prognosis and selecting appropriate treatments in patients with advanced MF/SS.
摘要：
背景：真菌病（MF）和Sezary综合征（SS）占所有皮肤T细胞淋巴瘤诊断的一半以上。当前的风险分层主要基于TNMB分期，很少有研究调查临床检查的预后价值。目前晚期疾病的全身治疗包括免疫调节药物，化疗,和HADC抑制剂。很少有临床试验或回顾性研究比较不同药物的疗效。方法：在这里，我们对2013-2023年在北京协和医院诊断为MF/SS的92例患者的预后因素和治疗结果进行了回顾性分析.结果：Cox回归分析确定年龄≥50岁，白细胞≥8×109/L,血清LDH≥250U/L,β2-MG≥4.50mg/L,IV期与总生存率降低有关，年龄≥50岁，血清LDH≥250U/L和IV期与无进展生存期降低相关.Kaplan-Meier分析确定免疫调节治疗与更长的无进展生存期相关。结论：这些结果为预测晚期MF/SS患者的预后和选择适当的治疗方法提供了新的因素。
