Cutaneous T-cell lymphoma

Last reviewed: 5 Jan 2023
Last updated: 20 Dec 2022

Summary

Definition

History and exam

Key diagnostic factors

  • skin patches, plaques, or tumors
  • poikiloderma
  • erythroderma
More key diagnostic factors

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
Other diagnostic factors

Risk factors

  • age >50 years
  • male gender
  • black ethnicity (MF); white ethnicity (SS)
  • exposure to infectious agents
  • chromosomal abnormality
  • environmental exposure to industrial chemicals, herbicides, pesticides
More risk factors

Diagnostic investigations

1st investigations to order

  • CBC
  • skin biopsy
  • clonal T-cell receptor rearrangement
  • flow cytometry
  • comprehensive metabolic panel
  • LFTs
  • serum lactate dehydrogenase
More 1st investigations to order

Investigations 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
More investigations to consider

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.

  • Cutaneous T-cell lymphoma images
  • Differentials

    • Psoriasis
    • Eczema
    • Dermatophyte infection
    More Differentials
  • Guidelines

    • NCCN clinical practice guidelines in oncology: T-cell lymphomas
    • NCCN clinical practice guidelines in oncology: hematopoietic cell transplantation
    More Guidelines
  • padlock-lockedLog in or subscribe to access all of BMJ Best Practice

Use of this content is subject to our disclaimer