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中国罕见病联盟皮肤罕见病专业委员会, 国家皮肤与免疫疾病临床医学研究中心, 中国医疗保健国际交流促进会皮肤科分会. 中国蕈样肉芽肿诊疗及管理专家指南[J]. 罕见病研究, 2023, 2(2): 191-209. doi: 10.12376/j.issn.2097-0501.2023.02.008
引用本文: 中国罕见病联盟皮肤罕见病专业委员会, 国家皮肤与免疫疾病临床医学研究中心, 中国医疗保健国际交流促进会皮肤科分会. 中国蕈样肉芽肿诊疗及管理专家指南[J]. 罕见病研究, 2023, 2(2): 191-209. doi: 10.12376/j.issn.2097-0501.2023.02.008
Rare Skin Disease Committee of China Alliance for Rare Diseases, National Clinical Research Center for Dermatologic and Immunologic Diseases, Dermatology Branch of China International Exchange and Promotion Association for Medical and Health Care. Expert Guidelines for the Diagnosis, Treatment, and Management of Mycosis Fungoides in China[J]. Journal of Rare Diseases, 2023, 2(2): 191-209. doi: 10.12376/j.issn.2097-0501.2023.02.008
Citation: Rare Skin Disease Committee of China Alliance for Rare Diseases, National Clinical Research Center for Dermatologic and Immunologic Diseases, Dermatology Branch of China International Exchange and Promotion Association for Medical and Health Care. Expert Guidelines for the Diagnosis, Treatment, and Management of Mycosis Fungoides in China[J]. Journal of Rare Diseases, 2023, 2(2): 191-209. doi: 10.12376/j.issn.2097-0501.2023.02.008

中国蕈样肉芽肿诊疗及管理专家指南

doi: 10.12376/j.issn.2097-0501.2023.02.008
基金项目: 

中央高水平医院临床科研业务费 2022-PUMCH-C-021

国家自然科学基金 82173449

北京市自然科学基金 7232114

中国医学科学院医学与健康科技创新工程 2022-I2M-C&T-A-007

通信作者:刘洁, E-mail:Liujie04672@pumch.cn 晋红中, E-mail:jinhongzhong@263.net
详细信息
  • 中图分类号: R739.5

Expert Guidelines for the Diagnosis, Treatment, and Management of Mycosis Fungoides in China

Funding: 

National High Level Hospital Clinical Research Funding 2022-PUMCH-C-021

National Natural Science Foundation of China 82173449

Beijing Natural Science Foundation 7232114

Chinese Academy of Medical Sciences(CAMS) Innovation Fund for Medical Sciences 2022-I2M-C&T-A-007

Corresponding authors: LIU Jie, E-mail: Liujie04672@pumch.cn JIN Hongzhong, E-mail: jinhongzhong@263.net
  • 摘要: 蕈样肉芽肿(MF)是原发于记忆性辅助T细胞的皮肤淋巴瘤。典型的MF皮损具有典型的3期表现,早期为斑片,逐渐形成浸润性斑块,晚期可出现肿瘤、红皮病,也可能累及淋巴结和其他脏器,自然病程可达数十年。MF早期诊断困难,需要定期随访、重复活检,并结合临床表现、组织病理、免疫表型、分子生物学进行综合判断。MF患者的治疗应根据分期决定,早期MF可选择外用药物、光疗、放疗等皮肤定向治疗,晚期MF或复发难治性早期MF需进行系统治疗,系统治疗均可联合皮肤定向治疗以缓解症状。多学科诊疗模式对于MF患者的管理十分重要,在制订治疗方案时应由多学科诊疗团队对患者进行整体评估,充分权衡可能的获益、患者耐受性及治疗副反应,以延缓疾病进展,改善患者生活质量。本指南参考国外国内的最新研究成果,结合中国的实际情况和专家经验制定,旨在规范中国MF的诊断、治疗与管理流程。

     

  • 图  1  MF治疗方案

    “ ”所指方格表示该阶段治疗可选择的药物种类,方格从上至下表示优先级别依次递减;“”所指方格表示一线治疗效果不佳时可选择的二线治疗方案; SDT: 皮肤定向治疗; PUVA: 补骨脂素光化学疗法;NB-UVB: 窄谱中波紫外线;TSEBT: 全身皮肤电子束治疗;HDACi: 组蛋白去乙酰化酶抑制剂;ECP: 体外光分离疗法

    Figure  1.  Treatment protocols of MF

    图  2  MF管理路径

    Figure  2.  Management pathway of MF

    表  1  早期蕈样肉芽肿(MF)诊断标准*

    Table  1.   Algorithm for diagnosis of early mycosis fungoides(MF)

    标准 评分标准
    临床特征 主要标准:
    存在持续性和/或进行性的斑片和斑块
    次要标准:
    ·非暴露部位的病变
    ·病变的大小、形状各异
    ·皮肤异色症
    符合主要标准
    ·同时符合1项次要标准1分
    ·同时符合2项或3项次要标准2分
    组织病理学 主要标准:
    浅表淋巴细胞浸润
    次要标准:
    ·不伴海绵形成的亲表皮现象
    ·淋巴细胞异型性
    符合主要标准
    ·同时符合1项次要标准1分
    ·同时符合2项次要标准2分
    分子生物学 克隆性TCR基因重排 基因重排阳性1分
    免疫病理学 ·少于50%的T细胞表达CD2、CD3或CD5
    ·少于10%的T细胞表达CD7
    ·表皮细胞和真皮细胞在CD2、CD3、CD5或CD7的表达上存在不一致
    满足任意1项或以上者1分
    *转载已获许可,©2005 American Academy of Dermatology, Inc.
    下载: 导出CSV

    表  2  改良的严重程度加权评估工具(mSWAT)*

    Table  2.   Modified Severity Weighted Assessment Tool(mSWAT)

    部位 体表面积(%BSA) 皮损类型
    斑片1 斑块2 肿瘤3
    7
    2
    躯干前部 13
    上臂 8
    前臂 6
    5
    躯干后部 13
    臀部 5
    大腿 19
    小腿 14
    7
    腹股沟 1
    皮损总体表面积
    权重因子 ×1 ×2 ×4
    皮损总体表面积×权重因子
    mSWAT评分为各类型皮损得分总和;1斑片指任何大小的无浸润或非隆起性皮损,可能出现皮肤异色样改变;2斑块指任何大小的隆起或浸润性皮损,可能出现结痂、溃疡或皮肤异色样改变;3肿瘤指直径≥1 cm的实性或结节样皮损,且有证据证明皮损浸润程度较深和/或垂直生长; *转载已获许可,©2011 American Society of Clinical Oncology
    下载: 导出CSV

    表  3  MF的TNMB分期*

    Table  3.   TNMB classification of MF

    分期 临床特征
    皮肤(T)
      T1 局限斑片,丘疹,和/或斑块,累及<10%体表面积,可以进一步分为T1a(仅有斑片)和T1b(斑块±斑片)
      T2 斑片,丘疹,和/或斑块,累及≥10%体表面积,可以进一步分为T2a(仅有斑片)和T2b(斑块±斑片)
      T3 一个或一个以上肿瘤(直径≥1 cm)
      T4 红斑融合,累及≥80%体表面积
    淋巴结(N)
      N0 无临床异常的外周淋巴结,无需活检
      N1 临床异常外周淋巴结,组织病理Dutch分级1或NCI LN0~2
        N1a 克隆阴性
        N1b 克隆阳性
      N2 临床异常外周淋巴结,组织病理Dutch分级2或NCI LN3
        N2a 克隆阴性
        N2b 克隆阳性
      N3 临床异常外周淋巴结,组织病理Dutch分级3~4或NCI LN4,克隆阴性或阳性
      NX 临床异常外周淋巴结,无组织病理证据
    内脏(M)
      M0 无内脏受累
      M1 内脏受累(必须由组织病理确诊,并且器官受累必须是特异性的)
    血液(B)
      B0 无明显血液受累:外周血中Sézary细胞≤5%
        B0a 克隆阴性
        B0b 克隆阳性
      B1 低血液肿瘤负荷,外周血中Sézary细胞>5%,但不满足B2的标准
        B1a 克隆阴性
        B1b 克隆阳性
      B2 高血液肿瘤负荷:克隆阳性且满足以下一项:Sézary细胞≥1000/μL;CD4/CD8≥10;CD4+CD7细胞≥40%或CD4+CD26细胞≥30%
    *转载已获许可,©2007 American Society of Hematology
    下载: 导出CSV

    表  4  MF临床分期系统*

    Table  4.   Clinical staging of MF

    临床分期 皮肤(T) 淋巴结(N) 内脏(M) 血液(B)
    ⅠA 1 0 0 0,1
    ⅠB 2 0 0 0,1
    ⅡA 1,2 1,2 0 0,1
    ⅡB 3 0~2 0 0,1
    ⅢA 4 0~2 0 0
    ⅢB 4 0~2 0 1
    ⅣA1 1~4 0~2 0 2
    ⅣA2 1~4 3 0 0~2
    ⅣB 1~4 0~3 1 0~2
    *转载已获许可,©2007 American Society of Hematology
    下载: 导出CSV

    表  5  MF淋巴结组织病理分期*

    Table  5.   Histopathologic staging of lymph nodes in MF

    ISCL/EORTC分期 Dutch分级 NCI-VA分期
    N1 1级:皮病性淋巴结炎 LN0:无异型淋巴细胞
    LN1:偶见或散在异型淋巴细胞(非簇集分布)
    LN2:多数异型淋巴细胞或呈3~6个细胞簇
    N2 2级:皮病性淋巴结炎;MF早期累及(存在脑回样核>7.5 μm) LN3:淋巴细胞聚集,淋巴结结构保留
    N3 3级:淋巴结结构部分破坏;多数异型性脑回状单一核细胞 LN4:异型淋巴细胞或肿瘤细胞部分或完全破坏淋巴结结构
    4级:完全破坏淋巴结结构
    *转载已获许可,©2007 American Society of Hematology
    下载: 导出CSV

    表  6  证据等级(牛津循证医学中心2011版)

    Table  6.   Oxford Centre for Evidence-Based Medicine 2011 Levels of Evidence

    证据等级 描述
    证据等级1 随机试验或n-of-1试验的系统综述
    证据等级2 随机试验或效果显著的观察性研究
    证据等级3 非随机对照队列或随访研究
    证据等级4 病例系列、病例对照研究或历史对照研究
    证据等级5 基于机制的推断
    下载: 导出CSV

    表  7  Fitzpatrick皮肤类型与推荐窄谱中波紫外线(NB-UVB)剂量*

    Table  7.   Treatment recommendations for dose of narrow band ultraviolet B(NB-UVB) based on Fitzpatrick skin type

    皮肤类型 起始剂量(mJ/cm2) 增加剂量(mJ/cm2)
    130 15
    220 25
    260 40
    330 45
    350 60
    400 65
    *转载已获许可,©2015 American Academy of Dermatology, Inc.
    下载: 导出CSV

    表  8  Fitzpatrick皮肤类型与推荐补骨脂素光化学疗法(PUVA)剂量*

    Table  8.   Treatment recommendations for dose of psoralen ultraviolet A (PUVA) based on Fitzpatrick skin type

    皮肤类型 起始剂量(J/cm2) 增加剂量(J/cm2)
    0.5 0.5
    1.0 0.5
    1.5 1.0
    2.0 1.0
    2.5 1.5
    3.0 1.5
    *转载已获许可,©2015 American Academy of Dermatology, Inc.
    下载: 导出CSV

    表  9  MF总体反应评价标准*

    Table  9.   Global response score of MF

    总体反应 皮肤 淋巴结 内脏 血液
    CR CR 均为CR或无受累(noninvolved,NI)
    PR CR 均无CR/NI,且均无PD
    PR 均无PD,如基线存在受累,至少一项为CR或PR
    SD PR 均无PD,如基线存在受累,均无CR或PR
    SD 各项为CR/NI、PR、SD,均无PD
    PD 任何一项为PD
    复发 任何一项为复发
    *转载已获许可,©2011 American Society of Clinical Oncology
    下载: 导出CSV
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  • 收稿日期:  2023-01-02
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