Last reviewed: 5 Oct 2024
Last updated: 20 Sep 2024
Summary
Definition
History and exam
Key diagnostic factors
- skin patches, plaques, or tumors
- poikiloderma
- erythroderma
Full details
Other diagnostic factors
- 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
Full details
Risk factors
- age >50 years
- male sex
- black ethnicity (MF); white ethnicity (SS)
- exposure to infectious agents
- ultraviolet light exposure
Full details
Diagnostic tests
1st tests to order
- CBC
- skin biopsy
- clonal T-cell receptor rearrangement
- flow cytometry
- comprehensive metabolic panel
- LFTs
- serum lactate dehydrogenase
Full details
Tests to consider
- 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
Full details
Treatment algorithm
ONGOING
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)
Contributors
Authors
Robert A. Schwartz, MD, MPH
Professor and Head
Department of Dermatology
Rutgers New Jersey Medical School
Newark
NJ
Disclosures
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
Disclosures
WCL declares he has no competing interests
Acknowledgements
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.
Disclosures
TMI, RC, and EP all declare that they have no competing interests.
Peer reviewers
Chris Kelsey, MD
Assistant Professor
Department of Radiation Oncology
Duke University School of Medicine
Durham
NC
Disclosures
CK declares that he has no competing interests.
Differentials
- Psoriasis
- Eczema
- Dermatophyte infection
More DifferentialsGuidelines
- NCCN clinical practice guidelines in oncology: T-cell lymphomas
- NCCN clinical practice guidelines in oncology: hematopoietic cell transplantation
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