小结
定义
病史和体格检查
关键诊断因素
- skin patches, plaques, or tumors
- poikiloderma
- erythroderma
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
危险因素
- age >50 years
- male sex
- black ethnicity (MF); white ethnicity (SS)
- exposure to infectious agents
- ultraviolet light exposure
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
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
治疗流程
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
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.
鉴别诊断
- Psoriasis
- Eczema
- Dermatophyte infection
更多 鉴别诊断指南
- NCCN clinical practice guidelines in oncology: T-cell lymphomas
- NCCN clinical practice guidelines in oncology: hematopoietic cell transplantation
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