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Cutaneous T-cell lymphoma

最后审阅: 18 Jun 2025
最后更新: 20 Sep 2024

小结

定义

病史和体格检查

关键诊断因素

  • skin patches, plaques, or tumors
  • poikiloderma
  • erythroderma
完整详情

其他诊断因素

  • pruritus
  • hypopigmented/hyperpigmented skin lesions
  • unilesional acral site involvement
  • lymphadenopathy
  • constitutional symptoms
  • palmar-plantar keratoderma
  • alopecia
  • leonine facies
  • onychodystrophy
  • hepatomegaly
  • ectropion
  • bullous, granulomatous, ichthyosiform, and purpuric lesions
完整详情

危险因素

  • age >50 years
  • male sex
  • black ethnicity (MF); white ethnicity (SS)
  • exposure to infectious agents
  • ultraviolet light exposure
完整详情

诊断性检查

首要检查

  • CBC
  • skin biopsy
  • clonal T-cell receptor rearrangement
  • flow cytometry
  • comprehensive metabolic panel
  • LFTs
  • serum lactate dehydrogenase
完整详情

需考虑的检查

  • screen for Sézary cells on blood film
  • human T-cell lymphotropic virus (HTLV)-I/2 serology
  • bone marrow biopsy
  • lymph node biopsy
  • CT scan or PET
  • HIV test
完整详情

治疗流程

持续性治疗

stage IA disease: limited skin involvement alone <10% body surface area (without large cell transformation)

stage IB to IIA disease: skin disease only with ≥10% body surface area (without large cell transformation)

stage IIB disease: tumor disease and no erythroderma (without large cell transformation)

stage III disease: erythrodermic (without large cell transformation)

stage IV disease: Sézary syndrome stage IVA1 or IVA2 (without large cell transformation)

stage IV disease: non-Sézary syndrome stage IVA2 or visceral disease/solid organ IVB (without large cell transformation)

large cell transformation

stage IIB, III, IV disease: refractory to multiple previous therapies (without large cell transformation)

撰稿人

作者

Robert A. Schwartz, MD, MPH

Professor and Head

Department of Dermatology

Rutgers New Jersey Medical School

Newark

NJ

利益声明

RAS declares that he has no competing interests.

W. Clark Lambert, MD, PhD

Professor and Associate Head, Dermatology

Director, Dermatopathology

New Jersey Medical School

Newark

NJ

利益声明

WCL declares he has no competing interests

鸣谢

Professor Robert A. Schwartz and Professor W. Clark Lambert would like to gratefully acknowledge Professor Tim M. Illidge, Dr Richard Cowan, and Dr Eileen Parry, previous contributors to this topic.

利益声明

TMI, RC, and EP all declare that they have no competing interests.

同行评议者

Chris Kelsey, MD

Assistant Professor

Department of Radiation Oncology

Duke University School of Medicine

Durham

NC

利益声明

CK declares that he has no competing interests.

参考文献

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

关键文献

Gilson D, Whittaker SJ, Child FJ, et al. Joint British Association of Dermatologists and UK Cutaneous Lymphoma Group guidelines for the management of primary cutaneous T-cell lymphomas. Br J Dermatol. 2019 Mar;180(3):496-526.全文  摘要

Willemze R, Hodak E, Zinzani P, et al; ESMO Guidelines Working Group. Primary cutaneous lymphomas: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2018 Oct 1;29(4 suppl):iv30-40.全文  摘要

National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: primary cutaneous lymphoma [internet publication].全文

参考文献

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.

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