Mycosis fungoides

Last Updated: 2022-02-25

Author(s): Anzengruber, Navarini

ICD11: 2B01

MF

Most common primary cutaneous T-cell lymphoma with low malignancy.

  • Incidence: 0,4-0,5/100.000/year
  • Occurring from the age of 40
  • Men : women = 2 : 1
  • Main forms
    • Folliculotropic MF
    • Pagetoid reticulosis
    • Granulomatous slack skin
  • Other forms
    • Syringotropic MF
    • Ichtyhyosiform MF
    • Pustular MF
    • Interstitial Mf
    • Vegetative papillomatous MF
    • Bullous MF
    • Hyperkeratotic verrucous MF
    • Erythrodermic mycosis fungoides
    • Palmoplantar MF
    • Poikilodermatitic MF
    • Hyper- and hypopigmented mycosis fungoides

Persistent stimulation from viruses, bacteria or other external influences is suspected as the cause. There is stimulation and proliferation of a lymphocyte clone with chromosomal instability.

  • Mostly the trunk and the flexor sides of the legs and arms are affected
  • Patch stage
    • Erythematous, sharply circumscribed, pityriasiform or psoriasiform, sometimes confluent macules
  • Plaque stage
    • Erythematous to purplish-brownish, lichenified plaques, sometimes associated with alopecia
  • Tumour stage
    • Exophytic, sometimes fungal, erythematous-livid tumours. Often shows erythroderma
  • Anamnesis
    • Duration?
    • Temporal progression?
    • B-symptomatology?
  • Clinic
    • Palpation of all lymph node stations, liver and spleen
  • Laboratory
    • ESR/CRP, differential blood count (often lymphocytosis and eosinophilia), liver enzymes, creatinine, LDH, electrolytes
    • FACS analysis, CD4/CD8 ratio, determination of CD4+CD7- cells
      • Immunelectrophoresis if necessary
      • If necessary, HTLV serology (especially for patients from abroad)
      • If necessary, Borrelia serology
    • Frequently elevated
  • Lymphocyte differentiation
    • FACS analysis: CD4+ cells↑, sometimes CD8+ cells↑
  • Sézary -cells (cells with large, indented nucleus of 6-10 μm size and in electron microscopy cerebriform nucleus and glycogen granules in the cytoplasm)?
    • At > 1000 cells/μL à Sézary syndrome
  • Biopsy
    • Immune phenotyping
    • Molecular biology: clonal rearrangment of T-cell receptor genes

Staging see latest SOP.

Patch, plaque stage:

  • Parakeratosis, possibly acanthosis, exocytosis, atypical lymphocytes with cerebriform nuclei, epidermotropism, Pautrier microabscesses and "lining up" at the dermal-epidermal junction.

Tumour stage:

  • Nodular atypical lymphocytic infiltrates. Epidermotropism and Pautrier microabscesses uncommon
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