Primär kutanes anaplastisches grosszelliges 30-positives Lymphom (solitäre oder lokalisierte Läsionen)

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%

• Bei isoliertem Tumor Exzision oder Bestrahlung (Fraktionierte Radiotherapie (2-3mal/Woche, GD 25-30 Gy, ED 2-5 Gy))
• Bei multifokalen Läsionen mit Regressionen: Bestrahlungstherapie (s.o.)
• Bei disseminiertem Auftreten: Chemotherapie oder Bestrahlungstherapie:
  • Chemotherapie:
    • Methotrexat
    • Knospe-Schema
    • CHOP-Schema
    • COPBLAM-Schema
    • Alternativ bei disseminierten Tumoren Ganzkörperbestrahlung mit schnellen Elektronen (GD: 30 Gy, ED: 2 Gy)

• 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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