Mycosis fungoides

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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