Mycosis fungoides

Letztmals aufdatiert: 2021-10-13

Autor(en): Anzengruber

MF

Häufigstes primär kutanes T-Zell-Lymphom mit niedriger Malignität.

  • Inzidenz: 0,4-0,5/100.000/Jahr
  • Ab dem 40 Lj. auftretend
  • Männer : Frauen = 2 : 1
  • Hauptform
  • Varianten:
    • Follikulotrope MF
    • Pagetoide Retikulose
    • Granulomatous slack skin
  • Seltene Varianten:
    • Syringotrope MF
    • Ichtyhyosiforme MF
    • Pustulöse MF
    • Interstitielle Mf
    • Vegetierend-papillomatöse MF
    • Bullöse MF
    • Hyperkeratotisch-verruköse MF
    • Erythrodermische Mycosis fungoides
    • Palmoplantare MF
    • Poikilodermatitische MF
    • Hyper- und hypopigmentierte Mycosis fungoides

Die Pathogenese ist weitgehend unklar. Eine mögliche Theorie ist die persistierende Stimulation durch Viren, Bakterien oder anderen externen Einflüssen. Dadurch komme es zu einer Stimulation und Proliferation eines Lymphozytenklons mit chromosomaler Instabilität.

  • Vor allem der Rumpf sowie die Beugeseiten der Beine und Arme sind betroffen
  • Patch-Stadium
    • Erythematöse, scharf begrenzte, pityriasiforme oder psoriasiforme, teils konfluierende Makulae
  • Plaque-Stadium
    • Erythematöse bis violett-bräunliche, lichenifizierte, teils mit einer Alopezie einhergehende Plaques
  • Tumor-Stadium
    • Exophytisch, teils pilzförmige, erythematös-livide Tumoren. Oftmals zeigt sich eine Erythrodermie
  • Anamnese
    • Dauer?
    • Zeitliche Progression
    • B-Symptomatik?
  • Klinik
    • Palpation aller Lymphknotenstationen, Leber und Milz. 
  • Labor
    • BSG/CRP, Differentialblutbild (oft Lymphozytose und Eosinophilie), Leberenzyme, Kreatinin, LDH, Elektrolyte
    • FACS-Analyse, CD4/CD8 Ratio, Bestimmung der CD4+CD7- Zellen
      • ggf. Immunelektrophorese
      • ggf. HTLV-Serologie (v.a. bei Pat. aus dem Ausland)
      • ggf. Borrelienserologie
    • IgE-Spiegel
      • Häufig erhöht
    • Lymphozytendifferenzierung
      • FACS-Analyse: CD4+-Zellen↑, manchmal CD8+ Zellen↑
    • Sézary -Zellen (Zellen mit großem, eingebuchtetem Kern von 6–10 μm Größe und in Elektronenmikroskopie Zerebriformer Kern sowie Glykogengranula im Zytoplasma)?
      • Bei > 1000 Zellen/μL starker Hinweis auf Sézary-Syndrom
  • Biopsie
    • Falls nur eine Biopsie, sollte die am stärksten indurierte Stelle gewählt werden
    • Immunphänotypisierung
    • Molekularbiologisch: Klonales Rearrangment von T Zell-Rezeptor-Genen.

Patch-, Plaquestadium:

  • Parakeratose, ggf. Akanthose, Exozytose, atypische Lymphozyten mit zerebriformen Kernen, Epidermotropismus, Pautrier-Mikroabszesse und "lining up" an der dermalen-epidermalen Junktion.

Tumorstadium:

  • Noduläre atypisch lymphozytäre Infiltrate. Epidermotropismus und Pautrier-Mikroabszesse ungewöhnlich

Häufig überlagerte Infektionen, da durch die Erkrankung eine Immunsuppression entsteht. 

Keine möglich.

Stadienabhängig von sehr gut im Stadium I (kaum Unterschiede zu nicht betroffenen Personen) zu infaust bei III bis IV.

Dermatologie USB Standard, analog ESMO Guideline 

Stadium IA-IIA

  • Therapiestufe 1
    • Abwartende Haltung
    • Topische Steroide Klasse IV, Evidenz A
    • UVBnb / PUVA
    • Topisches Mechlorethamin
    • Radiotherapie
  • Therapiestufe 2
    • SDT + Retinoide
    • SDT und IFN alpha
    • Retinoide
    • IFN alpha
    • Total skin electron beam therapy

Stadium IIB

  • Therapiestufe 1
    • SDT + lokale Radiotherapie
    • SDT + Retinoide
    • SDT und IFN alpha
    • Total skin electron beam therapy
  • Therapiestufe 2
    • Gemcitabin
    • Liposomales Doxorubicin
    • Brentuximab vedotin
    • Kombinationschemotherapie
    • Allogene Stammzelltransplantation

Stadium III

  • Therapiestufe 1
    • SDT + Retinoide
    • SDT + IFN alpha
    • ECP +/- IFN alpha +/- Retinoide
    • Low-dose Methotrexat
  • Therapiestufe 2
    • Total skin electron beam therapy

Stadium IV

  • Therapiestufe 1
    • Gemcitabin
    • Liposomales Doxorubicin
    • Brentuximab vedotin
  • Therapiestufe 2
    • Kombinationschemotherapie
    • Allogene Stammzelltransplantation

 

 

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