Diamandidou E, Colome-Grimmer M, Fayad L, Duvic M, Kurzrock R
Department of Bioimmunotherapy, Pathology, and Medical Specialties, University of Texas M.D. Anderson Cancer Center, Houston, TX 77030, USA.
Blood. 1998 Aug 15;92(4):1150-9.
The occurrence of large cell transformation has been well documented in a subgroup of patients with mycosis fungoides/Sezary syndrome (MF/SS). However, because of the rarity of MF/SS, little is known about the influence of clinicopathologic features in predicting large cell transformation and about outcome in the transformed cases. We evaluated all patients with MF/SS who were registered in our clinic during the study period and for whom pathologic slides for review were available or could be obtained. Disease was classified as transformed if biopsy showed large cells (>/=4 times the size of a small lymphocyte) in more than 25% of the infiltrate or if they formed microscopic nodules. Twenty-six patients with transformation were identified from a total of 115 evaluable cases with a diagnosis of MF/SS. The actuarial cumulative probability of transformation reached 39% in 12 years. The median time from diagnosis of MF/SS to transformation was 12 months (range, 0 to 128 months). Thirty-one percent of all patients with stage IIB-IV disease at presentation eventually transformed versus 14% of those with stage I-IIA (P = . 03), with transformation being especially common in patients with tumors (T3), 46% of whom transformed. Combining elevated beta2 microglobulin and lactic dehydrogenase (neither elevated v one or both elevated) was also predictive for transformation (P = .009). The median survival from initial diagnosis of MF/SS for the transformed patients was 37 months versus 163 months for the untransformed group (P = .0029). The median survival from transformation was 19.4 months (range, 2+ to 138 months). The following characteristics were associated with an inferior survival in transformed patients: (1) early transformation (<2 years from the diagnosis v >/=2 years; P = .011) and (2) advanced stage (IIB-IV v I-IIA; 2-year survival, 23% v 86%; P = .0035). We conclude that MF/SS patients with stages IIB-IV disease and, in particular, those with tumors have a high incidence of large-cell transformation. Patients with transformation have a relatively poor survival, especially if transformation occurs early (within 2 years) in the course of disease or if they are staged as IIB or higher.
大细胞转化在蕈样肉芽肿/赛塞里综合征（MF/SS）患者亚组中的发生已有充分记录。然而，由于MF/SS较为罕见，关于临床病理特征对预测大细胞转化的影响以及转化病例的预后情况，人们了解甚少。我们评估了研究期间在我们诊所登记的所有MF/SS患者，且有可供复查或能够获取的病理切片。如果活检显示超过25%的浸润中有大细胞（≥小淋巴细胞大小的4倍）或形成微小结节，则疾病被分类为已转化。在总共115例可评估的MF/SS诊断病例中，确定了26例发生转化的患者。12年时转化的精算累积概率达到39%。从MF/SS诊断到转化的中位时间为12个月（范围0至128个月）。初诊时处于IIB-IV期疾病的所有患者中，最终有31%发生转化，而处于I-IIA期的患者为14%（P = 0.03），转化在有肿瘤（T3）的患者中尤为常见，其中46%发生了转化。β2微球蛋白和乳酸脱氢酶升高（两者均未升高、仅一项升高或两者均升高）的联合情况也可预测转化（P = 0.009）。转化患者从MF/SS初始诊断后的中位生存期为37个月，而未转化组为163个月（P = 0.0029）。从转化开始的中位生存期为19.4个月（范围2 +至138个月）。以下特征与转化患者较差生存期相关：（1）早期转化（诊断后<2年对比≥2年；P = 0.011）和（2）晚期（IIB-IV对比I-IIA；2年生存率，23%对比86%；P = 0.0035）。我们得出结论，IIB-IV期疾病的MF/SS患者，尤其是有肿瘤的患者，大细胞转化发生率高。发生转化的患者生存期相对较差，特别是如果在疾病过程中早期（2年内）发生转化或分期为IIB期或更高。
