Primär kutanes diffuses grosszelliges B-Zell-Lymphom (isolierte Herde oder gruppierte Herde)

Letztmals aufdatiert: 2020-08-31

Autor(en): -

Primär kutanes diffus-grosszelliges B-Zell-Lymphom, leg type.

Zentroblastär und immunoblastär differenziertes, primär kutanes B-Zell Lymphom.

  • Häufigkeitsgipfel: 70-80 J.
  • Frauen > Männer

Assoziation: Rheumatoide Polyarthritis, chronische Lymphödemen

Meist an den Unterschenkeln auftretende erythematös, derb palpable, teils ulzerierte Knoten.

  • Klinik
  • Labor
  • BSG/CRP, Diff.-BB (Leukozytose, rel. Lymphozytose), Leber- und Nierenwerte, LDH, Elektrolyte
  • Borrelienserologie
  • Ggf. Immunelektrophorese
  • Ggf. HTLV-Serologie
  • Biopsie
  • Dermatopathologie
  • Immunhistologie: CD 19, CD20, CD79a, bcl-6 und CD10 pos. und meist bcl-2 neg.
  • Molekularbiologisch: PCR für die Immunglobulinkette
  • Lymphknotensonographie, Abdomensonographie, Röntgen-Thorax
  • Knochenmarksbiopsie optional

5-Jahresüberlebensrate: 40%

 

Ausdehnung

Therapie der ersten Wahl

Therapie der zweiten Wahl

Isolierte Herde oder gruppierte

Herde

Radiotherapie und/oder R-CHOP

Exzision

 

Multiple Herde

R-CHOP

Doxorubicin oder Gemcitabin,

ggf. in Kombination mit Rituximab

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

 

Bei Therapieresistenz:

  • Chimärer monoklonaler Anti-CD20-Antikörper
  • Rituximab i.v. 275 mg/m² KOF (Tag 0, 7, 14, 21)
  • Prämedikation:
    • Analgetika
    • 1000 mg Paracetamol p.o.

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