中华皮肤科杂志 ›› 2022, Vol. 55 ›› Issue (1): 20-26.doi: 10.35541/cjd.20210116

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伴大细胞转化的蕈样肉芽肿24例临床病理及免疫表型分析

张莹1    甘璐1    李思琪2    李颜2    宋昊1    邵雪宝1    张韡1    徐秀莲1    姜祎群1    曾学思1    陈浩1    孙建方1   

  1. 1中国医学科学院、北京协和医学院皮肤病医院病理科，南京  210042；2模式动物与疾病研究教育部重点实验室  医药生物技术国家重点实验室  化学和生物医药创新研究院  南京大学医学院模式动物研究所，南京  210061
  • 收稿日期:2021-02-02 修回日期:2021-10-11 发布日期:2021-12-31
  • 通讯作者: 陈浩；孙建方 E-mail:ch76ch@163.com; fangmin5758@aliyun.com
  • 基金资助:
    江苏省六大高峰人才项目（WSN-030）；南京市国家级临床医学中心培育计划项目（2019060001）；北京协和医学院研究生创新基金（3301030202030）；中国医学科学院医学与健康科技创新工程项目（CIFMS-2017-I2M-1-017）

Clinicopathological and immunophenotypic analysis of 24 cases of transformed mycosis fungoides

Zhang Ying1, Gan Lu1, Li Siqi2, Li Yan2, Song Hao1, Shao Xuebao1, Zhang Wei1, Xu Xiulian1, Jiang Yiqun1, Zeng Xuesi1, Chen Hao1, Sun Jianfang1   

  1. 1Department of Pathology, Hospital of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing 210042, China; 2The State Key Laboratory of Pharmaceutical Biotechnology, Chemistry and Biomedicine Innovation Center/Model Animal Research Center, Nanjing University, Nanjing 210061, China
  • Received:2021-02-02 Revised:2021-10-11 Published:2021-12-31
  • Contact: Chen Hao; Sun Jianfang E-mail:ch76ch@163.com; fangmin5758@aliyun.com
  • Supported by:
    Six Major Talent Summit in Jiangsu Province （WSN-030）; Nanjing Incubation Program for National Clinical Research Center （2019060001）; Peking Union Medical College Innovation Fund for Postgraduates （3301030202030）; CAMS Innovation Fund for Medical Sciences （CIFMS-2017-I2M-1-017）

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摘要： 【摘要】 目的 探讨伴大细胞转化的蕈样肉芽肿（TMF）的临床病理特征及预后。方法 回顾性分析2014—2020年中国医学科学院皮肤病医院诊治的24例TMF患者的临床病理资料及其中11例患者的16份外周血样本流式细胞术检测结果。结果 24例患者中男11例，女13例，诊断TMF时年龄平均为50.0（18 ~ 77）岁，早期（9例）及肿瘤期（15例）患者分别为44.8岁和52.6岁。诊断MF至发生大细胞转化的时间间隔平均为3.7年，其中8例初诊时即为TMF（经组织病理检查明确诊断）。组织病理显示，20例患者大细胞呈弥漫分布，4例呈灶状分布，其中7例大细胞比例 > 75%。免疫组化显示，18例患者表达CD30，其中9例CD30阳性大细胞比例 > 75%；6例不表达CD30。16份外周血样本（早期4份，肿瘤期12份）流式细胞仪检测结果显示，2份早期及10份肿瘤期样本中检测到表达克隆性TCR-vβ的细胞亚群，且16份样本均检测到前向角散射明显大于正常淋巴细胞的肿瘤细胞。随访结果显示，早期TMF患者中，3例发生大细胞转化后平均3.3年后进展为肿瘤期，1例进展为红皮病型MF（ⅢA期），另4例仍呈惰性病程；肿瘤期TMF患者中，1例进展为Ⅳ期，5例死亡，平均为发生大细胞转化后3.3（1.5 ~ 6）年。结论 大细胞转化可发生于任何疾病分期的MF患者，部分患者预后较差，应对TMF患者密切临床随访。

关键词: 蕈样真菌病, 细胞转化, 肿瘤, 皮肤表现, 病理过程, 诊断, 抗原, CD30, 大细胞转化

Abstract: 【Abstract】 Objective To investigate clinicopathological features and prognosis of transformed mycosis fungoides （TMF）. Methods A retrospective analysis was performed on clinicopathological data collected from 24 patients with TMF, as well as on flow cytometry results of 16 peripheral blood samples obtained from 11 of the 24 patients, who visited Hospital of Dermatology, Chinese Academy of Medical Sciences between 2014 and 2020. Results Among the 24 patients, 11 were males and 13 were females. Their average age at diagnosis of TMF was 50.0 years （range: 18 - 77 years）, and patients with early-stage TMF （9 cases） and tumor-stage TMF （15 cases） were aged 44.8 and 52.6 years on average, respectively. The average time interval from diagnosis of MF to large cell transformation was 3.7 years, and 8 patients were diagnosed with TMF at the initial visit. Histopathologically, large cells infiltrated in a diffuse pattern in 20 cases, as well as in a multifocal pattern in 4, and the proportion of large cells in 7 cases was greater than 75%. Immunohistochemically, 18 patients showed positive staining for CD30, and the proportion of CD30-positive large cells was greater than 75% in 9; negative staining for CD30 was observed in 6. Flow cytometry of 16 peripheral blood samples showed the presence of cell subsets expressing clonal T cell receptor （TCR）-vβ in 2 of 4 patients with early-stage TMF and 10 of 12 with tumor-stage TMF, and tumor cells with higher forward scatter than normal lymphocytes were detected in 16 samples. During the follow-up, among the patients with early-stage TMF, 3 progressed to tumor-stage TMF 3.3 years on average after large cell transformation, 1 progressed to erythrodermic MF in stage IIIA, and the other 4 still showed an indolent course; among the patients with tumor-stage TMF, 1 progressed to stage-IV TMF, and 5 died 3.3 （1.5 - 6） years after large cell transformation. Conclusion Large cell transformation may occur in patients with MF in any stage, some patients have poor prognosis, so close follow-up is needed for patients with TMF.

Key words: Mycosis fungoides, Cell transformation, neoplastic, Skin manifestations, Pathologic processes, Diagnosis, Antigens, CD30, Large cell transformation

引用本文

张莹 甘璐 李思琪 李颜 宋昊 邵雪宝 张韡 徐秀莲 姜祎群 曾学思 陈浩 孙建方. 伴大细胞转化的蕈样肉芽肿24例临床病理及免疫表型分析[J]. 中华皮肤科杂志, 2022,55(1):20-26. doi:10.35541/cjd.20210116

Zhang Ying, Gan Lu, Li Siqi, Li Yan, Song Hao, Shao Xuebao, Zhang Wei, Xu Xiulian, Jiang Yiqun, Zeng Xuesi, Chen Hao, Sun Jianfang. Clinicopathological and immunophenotypic analysis of 24 cases of transformed mycosis fungoides[J]. Chinese Journal of Dermatology, 2022, 55(1): 20-26.doi:10.35541/cjd.20210116