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

Zuletzt aktualisiert: 2022-11-16

Autor(en): -

ICD11: 2A81.A

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%

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