Vergier B, de Muret A, Beylot-Barry M, Vaillant L, Ekouevi D, Chene G, Carlotti A, Franck N, Dechelotte P, Souteyrand P, Courville P, Joly P, Delaunay M, Bagot M, Grange F, Fraitag S, Bosq J, Petrella T, Durlach A, De Mascarel A, Merlio J P, Wechsler J
Department of Pathology, Dermatology, and Statistics, University Hospital, Bordeaux, France.
Blood. 2000 Apr 1;95(7):2212-8.
The course of mycosis fungoides (MF) is indolent except when transformation to a large T-cell lymphoma occurs. The diagnosis of transformed MF (T-MF) relies on the presence of more than 25% of large cells on biopsy of an MF lesion. We analyzed 45 patients with T-MF recorded by the French Study Group on Cutaneous Lymphomas to better determine clinicopathological features of MF transformation and to analyze their impact on prognosis. Median time from diagnosis of MF to transformation was 6.5 years. Extracutaneous progression was present in 20 patients. Mean survival from transformation to death was 22 months. In univariate analysis, only an extracutaneous progression was associated with a worse prognosis (5-year actuarial survival: 7.8% versus 32%). Neither sex, age, clinical and skin disease stage at transformation, transformation speed, nor percentage of large cells or CD30 expression (14 of 45) had a prognostic value. When performing multivariate analysis, age (at least 60 years), and extracutaneous spreading were found to be associated with a poor prognosis. There was no difference between survival curves of patients with T-MF and with pleomorphic large T-cell CD30- lymphomas. The main diagnostic pitfall was "histiocytic-rich" MF, requiring CD68 staining for the diagnosis of T-MF. Out of 45 patients, 6 presented an histologic transformation before clinical progression, suggesting that an early histopathological diagnosis may be performed by histological follow-up. The prognostic value of such early histopathological diagnosis must be confirmed by prospective studies.
蕈样肉芽肿（MF）的病程通常较为缓慢，除非发生向大T细胞淋巴瘤的转化。转化型MF（T-MF）的诊断依赖于MF病变活检时大细胞比例超过25%。我们分析了法国皮肤淋巴瘤研究组记录的45例T-MF患者，以更好地确定MF转化的临床病理特征，并分析其对预后的影响。从MF诊断到转化的中位时间为6.5年。20例患者出现皮肤外进展。从转化到死亡的平均生存期为22个月。单因素分析中，只有皮肤外进展与较差的预后相关（5年精算生存率：7.8%对32%）。性别、年龄、转化时的临床和皮肤疾病分期、转化速度、大细胞百分比或CD30表达（45例中的14例）均无预后价值。多因素分析时，年龄（至少60岁）和皮肤外播散与预后不良相关。T-MF患者与多形性大T细胞CD30阴性淋巴瘤患者的生存曲线无差异。主要的诊断陷阱是“富含组织细胞”的MF，诊断T-MF需要进行CD68染色。45例患者中，6例在临床进展前出现组织学转化，提示通过组织学随访可进行早期组织病理学诊断。这种早期组织病理学诊断的预后价值必须通过前瞻性研究加以证实。
