Cutaneous T-cell lymphoma
Cutaneous T-cell lymphoma is a non-Hodgkin lymphoma of malignant CD4+ T cells that homes to the skin, most often as mycosis fungoides progressing through patch, plaque, and tumor stages, with Sezary syndrome as its aggressive leukemic, erythrodermic variant. Diagnosis rests on skin biopsy showing epidermotropic atypical lymphocytes with Pautrier microabscesses plus clonal T-cell receptor gene rearrangement, and management is staged, from skin-directed therapy in limited disease to systemic agents and extracorporeal photopheresis in advanced or leukemic disease.
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What this article covers
- Overview
Definition: cutaneous T-cell lymphoma is a non-Hodgkin lymphoma arising from malignant, clonal T cells that home to and proliferate within the skin, producing a chronic, often indolent eruption that can progress to systemic disease.
- Epidemiology
Most common cutaneous lymphoma: mycosis fungoides is the most frequently diagnosed subtype of cutaneous T-cell lymphoma.
- Risk factors
Chronicity of antecedent skin disease: a long-standing, nonspecific, relapsing dermatitis that resists standard treatment is the usual antecedent rather than an identifiable exposure.
- Pathophysiology
Clonal malignant T-cell proliferation: cutaneous T-cell lymphoma arises from a clonal population of malignant T cells, most often of the CD4+ (helper) memory phenotype, that infiltrates the skin.
- Clinical features
Mycosis fungoides, patch stage: nonspecific, often pruritic, scaly erythematous patches that vary in size, shape, and color, frequently misdiagnosed as eczema or psoriasis; pruritus is common and can be debilitating.
- Differential diagnosis
Atopic dermatitis: chronic pruritic eruption with flexural predominance, but classically begins in infancy or childhood and lacks a biopsy showing epidermotropic atypical lymphocytes (atopic dermatitis).
- Diagnosis
Diagnosis is established by skin biopsy showing epidermotropism of atypical lymphocytes with Pautrier microabscesses, confirmed by immunophenotyping and demonstration of a clonal T-cell population: a…
- Management
Confirm stage before treating: skin-limited (patch/plaque) disease is treated with skin-directed therapy; extensive plaque, tumor-stage, nodal, or blood involvement requires systemic therapy.
- Complications
Systemic dissemination: spread to lymph nodes and viscera (lung, liver, gastrointestinal tract) marks a major prognostic transition and shortens survival to less than 4 years.
- Prognosis
Stage is the dominant prognostic factor: disease confined to skin (patch or plaque stage) carries survival greater than 10 years, while extracutaneous involvement (nodal or visceral) reduces median survival to less than 4 years.
- Prevention
No primary prevention exists: there is no established modifiable risk factor or screening test that prevents cutaneous T-cell lymphoma from developing, so prevention focuses on early biopsy, staged…
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