Medical Oncology· Topic 12.7 Hematologic and HIV-associated malignancies· 12.7.7

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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