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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
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
作者姓名: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
作者单位: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
基金项目: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)。
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...

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
收稿时间:2021-02-02

Clinicopathological and immunophenotypic analysis of 24 cases of transformed mycosis fungoides
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
Authors:Zhang Ying  Gan Lu  Li Siqi  Li Yan  Song Hao  Shao Xuebao  Zhang Wei  Xu Xiulian  Jiang Yiqun  Zeng Xuesi  Chen Hao  Sun Jianfang
Affiliation: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
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.
Keywords:Mycosis fungoides  Cell transformation,neoplastic  Skin manifestations  Pathologic processes  Diagnosis  Antigens,CD30  Large cell transformation
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