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

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%

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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