Lymphom, primäres kutanes CD30+ anaplastisch-großzelliges Lymphom

Zuletzt aktualisiert: 2022-11-16

Autor(en): -

ICD11: 2B03.0

  • Sézary-Syndrom
  • Peripheres T-Zell-Lymphom

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%

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