Cutaneous T-cell lymphoma

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Last reviewed: September 2018

Last updated: May 2018

Summary

Diagnosis is based on clinical findings, skin biopsy, and laboratory blood tests, and usually requires specialist expertise.

Establishing a diagnosis can be challenging, as the condition can take many different forms in the skin: flat patches, raised plaques, large tumours, and/or marked erythroderma (intense and widespread reddening of the skin).

Early-stage disease is usually managed with skin-directed therapy (topical medications, phototherapy, and localised radiotherapy). If skin disease progresses, or the patient presents with advanced disease, systemic therapies are often necessary (chemotherapy, biological or immunological therapy, photopheresis).

Clinical trials may be considered in early and advanced disease. The choice of skin-directed therapy or systemic treatment is usually dependent on both doctor and patient preference, as no one treatment option has been shown to be superior to another.

Prognosis is closely related to the stage at diagnosis. Survival rates in early disease are excellent. However, although treatment may induce remission, patients are expected to relapse after a variable interval and are unlikely to be cured.

Definition

Cutaneous T-cell lymphomas (CTCL) make up a heterogeneous group of uncommon disorders characterised by clonal accumulation of T lymphocytes primarily or exclusively in the skin. Mycosis fungoides (MF) and its leukaemic variant, Sézary's syndrome (SS), are the most common subtypes. [1] MF frequently behaves as an indolent lymphoma with good prognosis for early-stage disease, while SS is considered a more aggressive form of the disease, associated with shortened survival.

History and exam

Key diagnostic factors

skin patches, plaques, or tumours
poikiloderma
erythroderma

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Other diagnostic factors

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

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Risk factors

age >60 years
male gender
black ethnicity (mycosis fungoides); white ethnicity (Sézary's syndrome)
exposure to infectious agents
chromosomal abnormality
environmental exposure to industrial chemicals, herbicides, pesticides

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Diagnostic investigations

1st investigations to order

FBC
screen for Sézary's cells on blood film
skin biopsy
polymerase chain reaction (PCR) for T-cell receptor
flow cytometry
basic metabolic panel
LFTs
serum LDH

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Investigations to consider

human T-cell lymphotropic virus (HTLV)-I serology
bone marrow biopsy
lymph node biopsy
CT scan
HIV test

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Emerging tests

FDG-PET scan

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Treatment algorithm

ONGOING

early disease

advanced disease

Contributors

Authors VIEW ALL 

Tim M. Illidge, MD, PhD, FRCR, MRCP, FRCPath

Professor of Targeted Therapy and Oncology

University of Manchester

Manchester Academic Health Sciences Centre

Manchester

UK

Disclosures

TMI declares that he has no competing interests.

Richard Cowan, MD, FRCR, MRCP

Consultant in Clinical Oncology

Christie NHS Foundation Trust

Manchester

UK

Disclosures

RC declares that he has no competing interests.

Eileen Parry, MD, MRCP

Consultant Dermatologist

Tameside Hospital Integrated Care Organisation NHS Trust

Manchester

UK

Disclosures

EP declares she has no competing interests.

Peer reviewers VIEW ALL 

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.

Robert A. Schwartz, MD, MPH

Professor and Head of Dermatology

Department of Medicine

New Jersey Medical School

Newark

NJ

Disclosures

RAS declares that he has no competing interests.

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