Department of Dermatology, Leiden University Medical Center, Leiden, The Netherlands.
Blood. 2012 Feb 16;119(7):1643-9. doi: 10.1182/blood-2011-08-376319. Epub 2011 Dec 12.
Large cell transformation (LCT) in mycosis fungoides (MF) is generally associated with an aggressive clinical course and poor survival, requiring aggressive therapeutic approach. However, a proportion of cases may follow an indolent clinical course. To identify prognostic factors, we analyzed the prognostic relevance of clinical, histologic, and immunophenotypical features in a large cohort of transformed MF patients, including 75 patients with only skin lesions, 19 patients with LCT in skin and lymph nodes, and 6 patients with LCT in lymph nodes only. Multivariate analysis of the total group showed that CD30 negativity, folliculotropic MF, extent of skin lesions and extracutaneous transformation were associated with reduced disease-specific survival (DSS) and, except for CD30 negativity and folliculotropic MF, also overall survival. In a multivariate analysis of 75 patients with only skin lesions at the time of LCT, CD30 negativity, folliculotropic MF and extent of skin lesions were independent parameters for both DSS and overall survival. Using the most discriminating parameters as a prognostic index, in both study groups differences in DSS between patients with 0-1 unfavorable prognostic factor(s) and ≥ 2 unfavorable prognostic factors were statistically significant (P < .001). This prognostic index may be helpful in predicting prognosis and selecting the most appropriate treatment in patients with transformed MF.
蕈样肉芽肿中出现大细胞转化（LCT）通常与侵袭性临床病程和较差的生存相关，需要积极的治疗方法。然而，一部分病例可能表现为惰性的临床病程。为了明确预后因素，我们分析了 75 例仅有皮肤病变、19 例皮肤和淋巴结均有 LCT、6 例仅有淋巴结受累的 LCT 患者的临床、组织学和免疫表型特征的预后相关性。总组的多变量分析显示，CD30 阴性、亲毛囊性蕈样肉芽肿、皮肤病变范围和皮肤外转化与疾病特异性生存（DSS）降低相关，除 CD30 阴性和亲毛囊性蕈样肉芽肿外，还与总生存相关。在仅有皮肤病变时出现 LCT 的 75 例患者的多变量分析中，CD30 阴性、亲毛囊性蕈样肉芽肿和皮肤病变范围是 DSS 和总生存的独立预后因素。使用最具鉴别性的参数作为预后指数，在两个研究组中，DSS 有 0-1 个不利预后因素和≥2 个不利预后因素的患者之间存在统计学差异（P<.001）。该预后指数有助于预测预后，并为 LCT 患者选择最合适的治疗方法。
