Primär kutanes anaplastisches grosszelliges 30-positives Lymphom (multifokale Läsionen rezidivierend)

Letztmals aufdatiert: 2020-08-31

Autor(en): Anzengruber, Navarini

ICD11: 2B03.0

Stein, 1985.

  • Häufigkeitsgipfel: Kinder, Jugendliche und auch im höheren Alter vorkommend
  • Männer > Frauen
  • Solitäre oder gruppiert auftretende, teils ulzerierte Knoten
  • Lymphknoten-, Lungen- und ZNS-Beteiligung möglich
  • Klinik
  • Labor
    • BSG/CRP, Diff.-BB (Leukozytose, rel. Lymphozytose), Leber- und Nierenwerte, LDH, Elektrolyte
    • FACS-Analyse, CD4/CD8 Ratio, Bestimmung der CD4+CD7- Zellen
    • Ggf. Immunelektrophorese
    • Ggf. HTLV-Serologie
    • Ggf. Borrelienserologie
  • Biopsie
    • Dermatopathologie
    • Immunhistologie
      • CD30+ von mindestens 75% der Tumorzellen, CD3+ und CD4+
      • ALK-1 negativ
    • Molekularbiologisch: Klonales Rearrangment von T-Zell-Rezeptor-Genen
  • Ganzkörper-CT, Lymphknotensonographie, ggf. PET-CT

Anaplastische und pleomorphe Zellen bilden noduläre dermale Infiltrate.

5-Jahres-Überleben: 90%

Ausdehnung

Therapie der 1. Wahl

Therapie der 2. Wahl
  • Solitäre oder lokalisierte Läsionen
  • Multifokale Läsionen rezidivierend
  • evtl. mit spontaner Remission
  • Exzision und/oder Radiotherapie
  • (PCALCL)
  • Beobachtung (LyP)
  • Beobachtung (LyP)
  • Low-dose Methotrexat
  • PUVA
  • IFN-α
  • Bexaroten
  • Gemcitabin

Stadler, R., et al., Short German guidelines: cutaneous lymphomas. J Dtsch Dermatol Ges, 2008. 6 Suppl 1: p. S25-31.

 

  • Biologicals
    • Anti-CD30-Antikörper
    • Brentuximab i.v. 1,8 mg/kg KG (max. 180 mg) alle 3 Wochen
    • Infusion über 30  Minuten
    • min. 8, max. 16 Zyklen
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