Kim Y H, Bishop K, Varghese A, Hoppe R T
Department of Dermatology, Stanford (Calif) University School of Medicine, USA.
Arch Dermatol. 1995 Sep;131(9):1003-8.
There are no large studies evaluating patients with erythrodermic mycosis fungoides and Sézary syndrome to determine the important prognostic factors that may influence survival. This is important since new treatment modalities have been proposed as superior to existing primary therapies. We performed a retrospective cohort study of 106 patients with erythrodermic mycosis fungoides and Sézary syndrome, followed up in the Stanford (Calif) Mycosis Fungoides Clinic, to define the important prognostic factors in this group.
Patients younger than 65 years have a more favorable survival profile than those 65 years or older (P < .005). Longer duration of symptoms before diagnosis ( > or = 10 years) tends to be associated with more favorable prognosis (p = .055). Lymph node stage is significantly correlated with survival; patients with overall stage III disease have more favorable prognosis than those with stage IV disease (P < .001). Patients with circulating Sézary cells in their blood have a significantly worse prognosis than those without (P < .005). Patient sex or race had no significant effect on overall survival outcome. Three distinct prognostic groups were identified, "favorable," "intermediate," and "unfavorable," according to the number of unfavorable prognostic factors (P < .005). The median survival in each group is 10.2, 3.7, and 1.5 years, respectively.
In patients with erythrodermic mycosis fungoides and Sézary syndrome, the important prognostic factors are patient age at presentation, the overall stage, and peripheral blood involvement. Survival varies widely, depending on these variables. These prognostic factors should be evaluated when analyzing survival and/or treatment efficacy data of these patients.
目前尚无大型研究对红皮病型蕈样肉芽肿和塞扎里综合征患者进行评估，以确定可能影响生存的重要预后因素。鉴于已提出新的治疗方式优于现有的一线治疗方法，这一点至关重要。我们对106例红皮病型蕈样肉芽肿和塞扎里综合征患者进行了一项回顾性队列研究，这些患者在斯坦福（加利福尼亚州）蕈样肉芽肿诊所接受随访，以确定该组患者的重要预后因素。
65岁及以下患者的生存情况优于65岁及以上患者（P <.005）。诊断前症状持续时间较长（≥10年）往往与更有利的预后相关（p =.055）。淋巴结分期与生存显著相关；III期疾病患者的预后优于IV期疾病患者（P <.001）。血液中存在循环塞扎里细胞的患者预后明显差于无循环塞扎里细胞的患者（P <.005）。患者性别或种族对总体生存结果无显著影响。根据不良预后因素的数量，确定了三个不同的预后组，即“有利”、“中等”和“不利”（P <.005）。每组的中位生存期分别为10.2年、3.7年和1.5年。
在红皮病型蕈样肉芽肿和塞扎里综合征患者中，重要的预后因素是就诊时的患者年龄、总体分期和外周血受累情况。生存情况因这些变量而有很大差异。在分析这些患者的生存和/或治疗疗效数据时，应评估这些预后因素。
