Long-term outcome of 525 patients with mycosis fungoides and Sezary syndrome - Clinical prognostic factors and risk for disease progression
Long-term outcome of 525 patients with mycosis fungoides and Sezary syndrome - Clinical prognostic factors and risk for disease progression
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DOI：
10.1001/archderm.139.7.857
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发表时间：
2003-07-01
影响因子：
--
通讯作者：
Hoppe, RT
中科院分区：
文献类型：
--
作者：
Kim, YH;Liu, HL;Hoppe, RT
目的：研究和更新真菌样霉菌病（MF）和Sezary综合征（SS）患者的临床特征和长期预后，并确定预测生存和疾病进展的重要临床因素。设计：单中心回顾性队列分析。单位：皮肤淋巴瘤学术转诊中心。患者：从1958年到1999年，在斯坦福大学皮肤淋巴瘤诊所对525名MF和SS患者进行了评估和管理。主要结果测量：我们通过Kaplan-Meier方法计算了长期精算总生存率和疾病特异性生存率以及疾病进展，并根据对照人群的预期生存率计算了生存的相对风险（RR）。结果：大多数患者表现为T1（30%）或T2（37%）疾病；18%表现为T3, 15%表现为T4受累。43%的死亡可归因于MF，主要发生在T3或T4患者中。T分型和临床分期越晚期的患者生存预后越差。除T1期或IA期患者外，MF患者死亡的RR高于对照人群（IB/IIA期RR为2.2，IIB/III期RR为3.9，IV期RR为12.8）。尽管IB期和IIA期患者的总生存期相似，但他们的疾病特异性生存期有显著差异（P = 0.006）。单变量分析中最重要的临床预后因素是患者年龄、TNM和B分类、总体临床分期分组以及有无皮外疾病。在多因素分析中，患者年龄、T分类和有无皮外疾病是最重要的独立因素。疾病进展到更晚期的TNM或B级、更差的临床分期或因MF而死亡的风险与初始T级的严重程度相关。发生皮外疾病的风险也与T分类相关，当检测到皮外疾病时，这些患者均未发生T1疾病。结论：MF和SS患者有不同的疾病进展或死亡风险。生存最重要的临床预测因素包括患者年龄、T分类和有无皮外疾病。不同临床分期之间显著的疾病特异性生存差异验证了美国国家癌症研究所目前MF临床分期系统的有效性。
Objectives: To study and update the clinical characteristics and long-term outcome of our patients with mycosis fungoides (MF) and Sezary syndrome (SS), and to identify important clinical factors predictive of survival and disease progression.Design: A single-center, retrospective cohort analysis.Setting: Academic referral center for cutaneous lymphoma.Patients: Five hundred twenty-five patients with MF and SS evaluated and managed at Stanford University Cutaneous Lymphoma Clinic, Stanford, Calif, from 1958 through 1999.Main Outcome Measures: We calculated long-term actuarial overall and disease-specific survivals and disease progression by the Kaplan-Meier method, and relative risk (RR) for survival calculated from expected survivals in control populations.Results: The majority of our patients presented with T1 (30%) or T2 (37%) disease; 18% presented with T3 and 15% with T4 skin involvement. Forty-three percent of deaths were attributable to MF, primarily in patients with T3 or T4 disease. The patients with a more advanced T classification and clinical stage had a worse survival outcome. Except for patients with T1 or stage IA disease, the RR for death is greater in patients with MF than in a control population (RR, 2.2 in stage IB/IIA disease, 3.9 in stage IIB/III disease, and 12.8 in stage IV disease). Despite similar overall survival in patients with stage IB or IIA disease, their disease-specific survivals were significantly different (P = .006). The most significant clinical prognostic factors in the univariate analysis were patient age, TNM and B classifications, overall clinical stage groupings, and the presence or absence of extracutaneous disease. In the multivariate analysis, patient age, T classification, and the presence of extracutaneous disease were the most important independent factors. The risk for disease progression to a more advanced TNM or B classification, worse clinical stage, or death due to MF correlated with the severity of the initial T classification. The risk for development of extracutaneous disease also correlated with T classifications none of these patients had T1 disease when their extracutaneous disease was detected.Conclusions: Patients with MF and SS have varying risks for disease progression or death. The most important clinical predictive factors for survival include patient age, T classification, and the presence of extracutaneous disease. The significant disease-specific survival differences between different clinical stages validate the usefulness of the present MF clinical staging system of the National Cancer institute.