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原发性皮肤T细胞淋巴瘤

Primary cutaneous T-cell lymphoma

ORPHA:171901疾病组中国目录 第2批 · 20

基本事实

患病率
1-9 / 100 000(Europe)

相关基因 2来自下位疾病

Orphanet 未在本条目上直接标注致病基因。下表由本组所属的下位疾病汇总而来,「来源条目」列给出基因实际标注在哪一个 ORPHA 条目上。

基因名称来源条目
HAVCR2hepatitis A virus cellular receptor 2ORPHA:86884
TNFRSF1BTNF receptor superfamily member 1BORPHA:2584

近两年的全球研究 103L2

2024/08 起在 Europe PMC 检索所得,按发表时间倒序显示最近 20 篇。标题未译成中文——自动翻译需要接入 LLM 服务,尚未引入。

  • 2026-07
    A digital polymerase chain reaction method targeting non-recombined T-cell receptor sequences aids in diagnosing primary cutaneous T-cell lymphomas
    Journal of Cancer · DOI · Europe PMC
  • 2026-06开放获取
    Mycosis Fungoides with Concurrent Squamous Cell Carcinoma in a Young Patient: A Rare Case
    Acta dermato-venereologica · DOI · Europe PMC
  • 2026-06
    Rare case of CD20-positive primary cutaneous T-cell lymphoma, NOS, with an aggressive clinical course
    Journal of clinical pathology · DOI · Europe PMC
  • 2026-06开放获取
    Granulomatous cutaneous T-cell lymphoma in a patient with atage IVB of mycosis fungoides: a case report and literature review
    Frontiers in oncology · DOI · Europe PMC
  • 2026-05开放获取
    Dose-de-escalated focal radiotherapy for primary cutaneous T-cell lymphoma: a lesion-based retrospective cohort study
    Acta oncologica (Stockholm, Sweden) · DOI · Europe PMC
  • 2026-05开放获取
    Peripheral T-Cell Lymphomas in Southern Taiwan: Epidemiology, Treatment Patterns, and Long-Term Outcomes
    EJHaem · DOI · Europe PMC
  • 2026-05开放获取
    Development and validation of a prognostic nomogram for overall survival in patients with primary cutaneous T-cell lymphoma: A SEER-based study
    Medicine · DOI · Europe PMC
  • 2026-04病例报告
    Chidamide maintenance after allogeneic hematopoietic stem cell transplantation in mycosis fungoides: A case report
    Transplant immunology · DOI · Europe PMC
  • 2026-04综述
    Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): A practical compartment-based review
    Blood reviews · DOI · Europe PMC
  • 2026-04开放获取
    Age-related histopathological and immunophenotypic variations in early-stage mycosis fungoides: a multicentre retrospective study in Türkiye
    BMJ open · DOI · Europe PMC
  • 2026-04
    Hypopigmented CD8 + Mycosis Fungoides in Sri Lanka: Clinicopathologic Evidence for an Indolent Variant
    The American Journal of dermatopathology · DOI · Europe PMC
  • 2026-04病例报告开放获取
    Mycosis Fungoides With Large Cell Lymphoma Transformation: A Report of a Rare Case
    The American journal of case reports · DOI · Europe PMC
  • 2026-04
    Results from a Pilot Study of HyBryte™ (Topical Synthetic Hypericin) Versus Valchlor<sup>®</sup> (Mechlorethamine) in the Treatment of CTCL
    Oncology and therapy · DOI · Europe PMC
  • 2026-04开放获取
    Molecular Markers Distinguishing Early-Stage Mycosis Fungoides From Atopic Dermatitis Skin Lesions
    Experimental dermatology · 被引 1 · DOI · Europe PMC
  • 2026-03综述开放获取
    Mimicry in Cutaneous Malignancy-Rare Forms of Mycosis Fungoides as Diagnostic Pitfalls: A Narrative Review
    Medicina (Kaunas, Lithuania) · DOI · Europe PMC
  • 2026-03开放获取
    ZNF460 inhibits HMGCL and promotes PI3K pathway in colon cancer
    Translational cancer research · DOI · Europe PMC
  • 2026-03开放获取
    Evaluation of immunohistochemical and gene expression of Janus kinase 1 and Janus kinase 3 in the skin of different clinical types of mycosis fungoides patients - Part II: reverse transcriptase-polymerase chain reaction
    Anais brasileiros de dermatologia · DOI · Europe PMC
  • 2026-03开放获取
    Prognostic factors in Sézary syndrome - a retrospective propensity score-matched study on 1277 patients
    Frontiers in immunology · DOI · Europe PMC
  • 2026-03病例报告开放获取
    Moderate-Dose Local Radiotherapy in Symptomatic CD30-Positive, Anaplastic Lymphoma Kinase (ALK)-Negative Primary Cutaneous T-cell Lymphoma
    Cureus · DOI · Europe PMC
  • 2026-03开放获取
    Evaluation of immunohistochemical and gene expression of Janus kinase 1 and Janus kinase 3 in the skin of different clinical types of mycosis fungoides patients - Part 1
    Anais brasileiros de dermatologia · DOI · Europe PMC

境外已获批用于本病的药物 4L2

欧盟 2 项、美国 2 项。同一药物在两地各批一次的,会分别列出。

「境外已获批」不等于「在中国能用」。中间隔着进口注册、临床急需境外新药通道、海南博鳌乐城国际医疗旅游先行区等几条路径,各有各的条件与费用。这一节能确定地告诉你的只有一件事:这个病在世界范围内已经有获得批准的药物,它叫什么名字。拿这个名字去问主治医生,是下一步最省力的做法。

药名一律保留英文原文,不作翻译——中国的药品通用名与英文名的音译经常不一致,译错会让人去找一个不存在的药。

已获孤儿药资格、尚未获批的在研药物(5 项)

孤儿药资格只是一种监管身份——它意味着监管机构认可这是罕见病用药并给予研发激励,不代表这个药已被证明有效,也不代表将来一定能上市。绝大多数最终不会成药。列在这里是为了看清有哪些方向正在被尝试。

  • Methotrexate with laurocapram美国1990-10-15
    Topical treatment of mycosis fungoides.
    官方记录
  • human anti-CD4 monoclonal antibody美国2004-08-13
    Treatment of mycosis fungoides
    官方记录
  • naloxone美国2010-11-23
    Topical treatment of pruritus associated with mycosis fungoides
    官方记录
  • MRG-106 oligonucleotide inhibitor of microRNA miR-155-5p美国2017-03-30
    Treatment of mycosis fungoides
    官方记录
  • priliximab美国2017-09-18
    Treatment of mycosis fungoides (MF)
    官方记录

数据来自欧洲药品管理局(EMA)的药品与孤儿药资格公开导出表,以及美国 FDA 孤儿药资格数据库。两边口径不同:欧盟一侧取的是当前状态仍为「已授权」的药品;美国一侧记录的是「曾获批准」这一事实,FDA 的公开表不追踪药物此后是否退市(例如 Relyvrio 于 2024 年撤市,表中仍记为已获批)。请以官方记录页为准。

在中国开展的临床试验 19L2

按病名在 ClinicalTrials.gov 检索、并校验研究中心含中国大陆而来。登记状态不等于现在真的能入组——务必按 NCT 号到原站核实,并与主治医生商量。

登记为可入组 10

  • 招募中NCT02848274
    ID Of Prognostic Factors In Mycosis Fungoides/Sezary Syndrome
    观察性 · 2016/10Stanford University
    中国研究中心 1 个:Beijing
  • 招募中NCT05518851
    TreAtments and outComes in paTients With prImary CutAneous Lymphoma
    观察性 · 2022/08/01Peking University First Hospital
    中国研究中心 5 个:Beijing、Guandong、Jinan、Nanjing
  • 招募中NCT06436677
    A Study of Molecular Subtyping-based Therapeutic Strategies for Cutaneous T-cell Lymphoma
    观察性 · 2024/05/09Peking University First Hospital
    中国研究中心 3 个:Beijing
  • 招募中NCT06716658
    JAK1 Inhibitor Golidocitnib for the Treatment of Relapsed/Refractory Indolent T/NK-cell Lymphomas
    II 期 · 干预性 · 2024/12/25Institute of Hematology & Blood Diseases Hospital, China
    中国研究中心 2 个:Tianjin
  • 招募中NCT06914037
    A Clinical Study of CHT101 in CD70-Positive Relapsed or Refractory Hematological Malignancies
    I 期 · 干预性 · 2025/03/31Tianjin Medical University Cancer Institute and Hospital
    中国研究中心 1 个:Tianjin
  • 招募中NCT06925464
    CD7 CAR T-Cell Therapy for r/r T-cell Lymphomas
    I 期、II 期 · 干预性 · 2025/04/01Beijing GoBroad Hospital
    中国研究中心 4 个:Beijing、Shanghai、Zhanjiang
  • 招募中NCT07022964
    CD5 CAR T-Cell Therapy for r/r T-cell Lymphomas
    I 期、II 期 · 干预性 · 2025/06/01Beijing GoBroad Hospital
    中国研究中心 3 个:Beijing、Shanghai
  • 招募中NCT07754890
    Golidocitinib and Chidamide for Cutaneous T-Cell Lymphoma
    I 期、II 期 · 干预性 · 2025/08/26Peking Union Medical College Hospital
    中国研究中心 1 个:Beijing
  • 尚未开始招募NCT07535710
    Aclarubicin Plus Cyclophosphamide, Vincristine, and Prednisone (CAOP) in Patients With Previously Treated Cutaneous T-cell Lymphoma
    I 期、II 期 · 干预性 · 2026/03/30Shanghai Jiao Tong University School of Medicine
    中国研究中心 2 个:Shanghai、Wuxi
  • 尚未开始招募NCT07717580
    Efficacy and Safety of Brentuximab Vedotin Combined With Lisaftoclax in CD30+ Cutaneous T-cell Lymphoma (CTCL)
    II 期 · 干预性 · 2026/07/31Peking University First Hospital
    中国研究中心 1 个:Beijing
其他状态的试验(9 项)
  • 已终止NCT02597153
    Phase II Clinical Study of PLM60 for the Treatment of Cutaneous T Cell Lymphomas
    II 期 · 干预性 · 2015/10CSPC ZhongQi Pharmaceutical Technology Co., Ltd.
    中国研究中心 1 个:Beijing
  • 已完成NCT03493451
    Study of BGB-A317 in Participants With Relapsed or Refractory Mature T- and NK-cell Neoplasms
    II 期 · 干预性 · 2018/04/13BeiGene
    中国研究中心 13 个:Beijing、Chengdu、Fuzhou、Guangzhou、Hangzhou、Harbin 等 10 地
  • 状态未知NCT03999697
    A Clinical Research of CD22-Targeted CAR-T in B Cell Malignancies
    I 期 · 干预性 · 2018/12/01PersonGen BioTherapeutics (Suzhou) Co., Ltd.
    中国研究中心 1 个:Hefei
  • 状态未知NCT04296786
    Sintilimab Plus Chidamide in the Treatment of Relapsed and Refractory Cutaneous T-cell Lymphoma: a Multicenter Phase II Study
    II 期 · 干预性 · 2019/11/01Peking Union Medical College Hospital
    中国研究中心 1 个:Beijing
  • 状态未知NCT05781386
    A Phase I Trial of SIM1811-03 in Subjects With Advanced Tumors
    I 期 · 干预性 · 2022/03/06Jiangsu Simcere Biologics Co., Ltd
    中国研究中心 1 个:Guanzhou
  • 已完成NCT05442554
    A Study of Brentuximab Vedotin Treatment in Chinese Adults With CD30-Positive Cutaneous T-Cell Lymphoma
    IV 期 · 干预性 · 2022/11/28Takeda
    中国研究中心 4 个:Beijing、Chengdu、Shanghai
  • 进行中·不再招募NCT06285370
    A Study to Evaluate the Efficacy and Safety of KW-0761 in Chinese Subjects With Mycosis Fungoides or Sézary Syndrome Previously Treated With Systemic Therapy
    IV 期 · 干预性 · 2023/05/29Kyowa Kirin China Pharmaceutical Co., Ltd.
    中国研究中心 8 个:Beijing、Chengdu、Guangzhou、Hangzhou、Henan、Hohhot 等 8 地
  • 状态未知NCT06037239
    Phase I/II Study of Linperlisib Plus Chidamide for R/R Cutaneous T-cell Lymphoma: a Prospective, Single-center Study
    I 期、II 期 · 干预性 · 2023/07/01Peking Union Medical College Hospital
    中国研究中心 1 个:Beijing
  • 进行中·不再招募NCT06485219
    A Phase IIa Clinical Study of Purinostat Mesylate for Injection in Patients With Peripheral T-Cell Lymphoma and Cutaneous T-Cell Lymphoma
    II 期 · 干预性 · 2024/04/14Chengdu Zenitar Biomedical Technology Co., Ltd
    中国研究中心 2 个:Chengdu、Shanghai

中国境外的在招试验 81L2

这些试验在中国没有研究中心,通常无法直接报名——入组一般要求在当地居住并接受随访。列在这里是因为它另有用处:看清楚全世界正在试哪些药、做到了哪一期、由谁在做。把药名和 NCT 编号记下来去问主治医生,或据此进一步查该药是否已在境外获批、是否有拓展性用药(expanded access)通道。

美国51意大利9西班牙5法国5澳大利亚3丹麦2韩国2德国2Costa Rica1比利时1荷兰1Moldova1波兰1罗马尼亚1另有 6 个国家/地区

CT.gov 报告命中 81 项,此处取回并展示最近的 15 项。

  • 尚未开始招募NCT07608549
    FLASH Pilot Phase 1 Safety Study
    不适用 · 干预性 · 2029/10/31Lesley A. Jarvis
  • 尚未开始招募NCT07691450
    Belinostat in Combination With Azacitidine or Pralatrexate for the Treatment of Relapse or Refractory T-cell Lymphoma
    I 期 · 干预性 · 2027/01/03City of Hope Medical Center
    美国
  • 尚未开始招募NCT05728879
    Characterization of the Microbiome in Cutaneous T Cell Lymphoma Skin Lesions Before and After Use of APR-TD011® (RLF-TD011®) Spray Solution
    早期 I 期 · 干预性 · 2027/01Northwestern University
    美国
  • 尚未开始招募NCT07714187
    A Study of Topical Duvelisib in Participants With Early-stage Mycosis Fungoides
    I 期 · 干预性 · 2027/01SecuraBio
    美国
  • 尚未开始招募NCT07710781
    Study to Characterize Mismatched to Fully HLA-Matched Ossium HPC, Marrow and Living Donor Transplantation in Patients With Hematologic Malignancies
    不适用 · 干预性 · 2026/12/01Ossium Health, Inc.
  • 尚未开始招募NCT07682792
    Golidocitinib in Patients With Mycosis Fungoides/Sézary Syndrome and T-Cell Large Granular Lymphocytic Leukemia
    II 期 · 干预性 · 2026/09/30Washington University School of Medicine
    美国
  • 尚未开始招募NCT07192471
    A First-in-Human Study of KK2223 in Participants With Relapsed or Refractory T Cell Non-Hodgkin Lymphoma
    I 期 · 干预性 · 2026/08Kyowa Kirin, Inc.
    意大利、西班牙、美国
  • 尚未开始招募NCT07529405
    A Study of VG712 in Patients With Mycosis Fungoides
    II 期 · 干预性 · 2026/07/30Virogen Biotechnology Inc.
    美国
  • 尚未开始招募NCT07658924
    Efficacy and Safety of Benvitimod Cream in Cutaneous T-Cell Lymphoma
    II 期 · 干预性 · 2026/07/01Peking University First Hospital
  • 尚未开始招募NCT07538960
    Epidemiology and Management of Cutaneous T-Cell Lymphoma at Hospital México, Costa Rica.
    观察性 · 2026/05Caja Costarricense de Seguro Social
    Costa Rica
  • 尚未开始招募NCT07477769
    Lymphocyte Phenotype of Autosomal Recessive Congenital Ichthyoses Mutated NIPAL4 (Nipal4-nEDD)
    观察性 · 2026/04/01Assistance Publique - Hôpitaux de Paris
  • 招募中NCT05544968
    Anti-CD30 biAb-AATC in Patients With Relapsed/Refractory CD30 Positive Hematopoietic Malignancies
    I 期 · 干预性 · 2026/03/04Medical College of Wisconsin
    美国
  • 招募中NCT07356245
    Ruxolitinib Maintenance Post-Hematopoietic Stem Cell Transplant T-Cell Lymphoma
    II 期 · 干预性 · 2026/02/12Jonathan Brammer
    美国
  • 招募中NCT07195916
    A Study to Evaluate INCA036873 in Participants With Advanced Solid Tumors and Hematological Malignancies
    I 期 · 干预性 · 2026/01/08Incyte Corporation
    澳大利亚、比利时、丹麦、意大利、美国
  • 尚未开始招募NCT07213882
    A Phase 1, Multicenter, Open-label, Prospective, First-in-human Dose-escalation Clinical Trial of Domain Therapeutics' Anti-CCR8 Monoclonal Antibody (DT-7012) in Patients With Relapsed or Refractory Cutaneous T-cell Lymphomas (CTCL)
    早期 I 期 · 干预性 · 2026/01/01Assistance Publique - Hôpitaux de Paris

外部标识与链接

发现这一页有错误?告诉我 · 邮件主题会自动带上本页的 ORPHA 编号

本页数据来源

  • 疾病定义、同义词、基因、表型、流行病学:Orphanet(CC BY 4.0)
  • 中文病名:Orphanet 中文包,冻结于 2020-06-01
  • 表型中文标签:HPO 简体中文翻译(CHPO 上游成果)