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Cutaneous T-Cell Lymphoma (Mycosis Fungoides)

Most common primary cutaneous lymphoma; an indolent CD4+ T-cell lymphoma evolving through patch, plaque, and tumor stages, occasionally progressing to systemic involvement (Sézary syndrome).

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dermatoloji department. Book Appointment →

What is Cutaneous T-Cell Lymphoma (Mycosis Fungoides)?

Mycosis fungoides (MF) is the most common subtype of primary cutaneous T-cell lymphoma (CTCL), accounting for about 50% of all cutaneous lymphomas. It is characterized by clonal proliferation of skin-homing CD4+ helper T cells with epidermotropism (Pautrier microabscesses).

Disease evolves through stages: patch (erythematous scaly patches in covered areas), plaque (infiltrated indurated plaques), tumor (nodules and ulcerations), and erythroderma. Sézary syndrome is the leukemic variant with erythroderma, lymphadenopathy, and circulating Sézary cells.

Diagnosis often requires repeated skin biopsies over months to years, with TCR gene rearrangement, immunohistochemistry (loss of CD7, CD26), and clinical-pathologic correlation. TNMB staging guides therapy with skin-directed treatments in early stages and systemic therapies for advanced disease.

Symptoms

Persistent erythematous, scaly patches in covered areas (buttocks, breasts, inner thighs)
Slowly progressive plaques that thicken and infiltrate
Pruritus often severe and refractory
Tumor stage with raised nodules and ulcerations
Erythroderma (>80% body surface area) in advanced disease
Lymphadenopathy in advanced stages
Sézary syndrome: erythroderma + lymphadenopathy + circulating Sézary cells

Risk Factors

Older age (median 55-60 years) with male predominance
African American ethnicity (higher risk and worse prognosis)
Chronic antigenic stimulation (atopic dermatitis, occupational exposure)
HTLV-1 infection (especially adult T-cell leukemia/lymphoma)
Immunosuppression and HIV infection
Genetic alterations (TP53, JAK/STAT pathway mutations)
Family history of lymphoma (rare)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Persistent erythematous patches/plaques resistant to topical steroids
  • Recurrent pruritic dermatitis lasting more than 6 months
  • New plaques or tumors arising in chronic eczema
  • Erythroderma with lymphadenopathy
  • Suspected Sézary syndrome (rapidly progressive erythroderma)
  • Need for repeat skin biopsies to confirm diagnosis
  • Consultation with dermatology/hematology for staging

Treatment Methods

01
Stage-based therapy per ISCL/EORTC guidelines
02
Early stage (IA-IIA): topical corticosteroids, topical mechlorethamine, narrowband UVB, PUVA, localized radiation
03
Advanced stage (IIB-IV): total skin electron beam therapy, systemic retinoids (bexarotene), interferon
04
Systemic chemotherapy or targeted agents (mogamulizumab, brentuximab vedotin, romidepsin, vorinostat)
05
Allogeneic hematopoietic stem cell transplantation in selected advanced cases
06
Phototherapy maintenance for chronic disease
07
Multidisciplinary follow-up with dermatology, hematology, oncology

Which Department to Visit?

You can visit our Dermatoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.