进展期蕈样肉芽肿临床病理特征分析  

Clinicopathological characteristics of advanced-stage mycosis fungoides

作　　者：李芬[1] 何林[1] 王一鸣[2] 曾彦 Li Fen;He Lin;Wang Yiming;Zeng Yan(Department of Pathology,Chengdu Second People's Hospital,Chengdu 610017,China;Department of Dermatology,Chengdu Second People's Hospital,Chengdu 610017,China;Department of Hematology,Chengdu Second People's Hospital,Chengdu 610017,China)

机构地区：[1]成都市第二人民医院病理科,成都610017 [2]成都市第二人民医院皮肤科,成都610017 [3]成都市第二人民医院血液科,成都610017

出　　处：《白血病.淋巴瘤》2025年第2期92-97,共6页Journal of Leukemia & Lymphoma

基　　金：成都市卫生健康委员会医学科研课题(2022620)。

摘　　要：目的探讨进展期蕈样肉芽肿(MF)的临床病理特征。方法回顾性病例系列研究。分析2015年1月至2023年7月成都市第二人民医院5例进展期MF患者临床资料,总结其临床病理特征。结果5例MF患者中,男性2例,女性3例;中位年龄55(45~86)岁;病史2~16年;主要症状为皮肤瘙痒、红斑,皮损表现为红斑、鳞屑、斑块、水疱、糜烂、溃疡、色素沉着、结节、红皮病。皮肤组织学呈银屑病样、界面皮炎、非感染性肉芽肿、深浅血管周围皮炎、肿瘤等多种模式。1例为大疱性MF,2例为红皮病型MF,1例为肉芽肿性MF,1例为经典型MF。4例出现淋巴细胞亲表皮,3例出现胞质空晕的细胞沿基底层排列和Pautrier微脓疡,1例发生大细胞转化;肿瘤细胞CD3、CD4均阳性,CD8、CD56、ALK、CD20均阴性,EBER 1/2原位杂交阴性,转化大细胞CD30阳性,T细胞受体基因重排均阳性。2例骨髓、外周血及1例脑脊液检出肿瘤细胞,1例头部磁共振成像提示右颞部异常信号结节,2例淋巴结结构完全破坏。TNMB分期ⅡB期2例,ⅣA2期2例,ⅣB期1例。2例未治疗,1例接受干扰素α为主的系统性治疗,2例接受化疗或联合鞘内注射、放疗等,均只获得部分缓解。1例发生心源性猝死,2例死于肺部感染,2例带瘤生存。结论进展期MF的皮损及组织学改变多样,多学科联合诊疗有助于诊断及治疗。Objective To investigate the clinicopathological features of advanced-stage mycosis fungoides(MF).Methods A retrospective case-series study was conducted.The clinical data of 5 cases diagnosed with advanced-stage MF in Chengdu Second People's Hospital between January 2015 and July 2023 were analyzed.The clinicopathological features of patients were summarized.Results There were 2 males and 3 females in 5 MF patients,with the median age of 55 years(45-86 years)and the medical history of 2-16 years.The main symptoms were pruritus and erythema.The lesions were presented by erythema,scales,plaques,blisters,erosion,ulcers,pigmentation,nodules,and erythroderma.Histopathological examination showed different skin lesion patterns such as psoriasis-like,interfacial dermatitis,non-infectious granuloma,deep and shallow perivascular dermatitis,tumors.Among 5 patients,1 case was mycosis fungoides bullosa,2 cases were erythrodermic MF,1 case was granulomatous MF,and 1 case was classical MF.Lymphocyte epidermis was found in 4 cases,cytoplasmic halos cells lined up along the basal layer of the epidermis and Pautrier microabscess were found in 3 cases,large-cell transformation was found in 1 case.Tumor cells were positive for CD3,CD4 and negative for CD8,CD56,ALK and CD20;EBER 1/2 hybridization in situ was negative.CD30 was positive in transformed large cells and T cell receptor gene rearrangement was positive.The tumor cells were detected in bone marrow and peripheral blood of 2 cases and in cerebrospinal fluid of 1 case.Head magnetic resonance imaging of 1 case indicated abnormal signal nodules in the right temporal region and the normal architecture of the lymph nodes in 2 cases was completed destroyed by malignant cells.TNMB stage:2 cases were in stageⅡB,2 cases were in stageⅣA2,and 1 case was in stageⅣB.Interferonα-based systemic therapy was performed in 1 case,2 cases received chemotherapy or combined with intrathecal injection and radiotherapy,and other 2 cases were not treated.All of them just achieved partial remis

关 键 词：淋巴瘤 T细胞 皮肤 蕈样真菌病 病理状态 体征和症状 

分 类 号：R73[医药卫生—肿瘤]