Mycosis fungoides

Mycosis fungoides 

Updated: 03/17/2021

© Jun Wang, MD, PhD

General features
  • Most common type of cutaneous T cell lymphoma
  • More commonly seen in adults/elderly, men, African
  • Unknown etiology
  • Clonal CD4+ T cells
  • Diagnosis based on clinical presentations, biopsy, molecular testing and immunopathologic profiling 
Clinical presentations
  • Four stages: Patch, plaque, tumoral and Sézary syndrome
  • Patch stage: Pruritic erythematous macules or patches with telangiectasia and atrophy, may disappear spontaneously
  • Plaque stage: Pruritic thichened plaques, may resemble psoriasis
  • Tumoral stage: Tumor formation, either from plaques or de novo, may ulcerate
  • Sézary syndrome
    • Commonly erythroderma (80% of total body surface), may be scaly
    • Lymphadenopathy
    • Sézary cells in skin, lymph nodes and peripheral blood
    • Usually do not evolve from patches, plaques or tumors
    • May have marrow involvement
Key morphological features
  • Patch stage: Psoriasiform changes, but intraepidermal clonal CD4+ T cells
  • Plaque stage: Dermal infiltrate of atypical T cells with cerebriform nuclei alone or clustered in epidermis and in small sheets in dermis
  • Tumoral stage: Dense dermal infiltrates of atypical T cells with cerebriform nuclei
  • Sézary syndrome: More monotonous infiltrates, tumor cells in peripheral blood
Markers
  • Positive: CD4
  • Negative: CD8
Other laboratory tests
  • T-cell receptor gene rearrangement
  • T-cell receptor gene sequencing
  • Flow cytometry (SS): CD4+CD7- T cell subset
Treatment
  • Early stage: Topical corticosteroids, chemotherapy, retinoids, radiation, phototherapy, etc
  • Advanced stages (IIB to IV): Local radiation, total skin electron beam therapy, systemic chemotherapy, phototherapy, interferon, retinoids, etc

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