Primary cutaneous anaplastic large cell CD30 positive lymphoma (recurrent multifocal lesions)

Last Updated: 2020-08-11

Author(s): Anzengruber, Navarini

ICD11: 2B03.0

Stein 1985.

  • Frequency peaks: children, adolescents and also occurring in older age groups.
  • Men > Women.
  • Solitary or grouped, partly ulcerated nodules.
  • Lymph node, lung and CNS involvement possible.

  • Clinical features

  • Laboratory

    • ESR/CRP, Diff.-Blood count (leukocytosis, relative lymphocytosis), liver and kidney values, LDH, electrolytes.

    • FACS analysis, CD4/CD8 ratio, determination of CD4+CD7 cells.

    • Immunoelectrophoresis, if necessary

    • HTLV serology, if necessary

    • Borrelia serology, if necessary

  • Biopsy

  • Dermatopathology

  • Immunohistology

    • CD30+ from at least 75% of tumor cells, CD3+ and CD4+ ALK-1 negative.

  • Molecular biology

    • Clonal rearrangement of T cell receptor genes.

  • Whole body CT, lymph node sonography, possibly PET-CT.

Anaplastic and pleomorphic cells form nodular dermal infiltrates. 

5-year survival rate: 90%. 

Expansion

1st choice therapy

2nd choice therapy
  • Solitary or localised lesions
  • Recurrent multifocal lesions
  • Possibly with spontaneous remission
  • Excision and/or radiotherapy
  • (PCALCL)
  • Observation (LyP)
  • Low-dose Methotrexate
  • PUVA
  • IFN-α
  • Bexarotene
  • Gemcitabine

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

 

  • Biologicals

  • Anti-CD30 antibodies

  • Brentuximab intravenously 1.8 mg/kg bw (max. 180 mg) every 3 weeks

  • Infusion over 30 minutes

  • min. 8, max. 16 cycles

 

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