What are the guidelines on the treatment of primary cutaneous CD30+ T-Cell lymphoproliferative disorders (CD30+LPDs)?

Updated: Feb 25, 2021
  • Author: Sanjay Vinjamaram, MD, MPH; Chief Editor: Emmanuel C Besa, MD  more...
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Both guidelines give similar treatment recommendations for PC-ALCL, as follows [26, 88] :

  • For solitary or grouped lesions, surgical excision or radiation therapy for first-line treatment

  • Relapses are equally frequent after both treatments; if confined to the skin, treatment can be repeated

  • For multifocal lesions, low-dose methotrexate may be used, based on expert consensus; retinoids and interferon alpha are alternative treatments for multifocal disease that is not responsive to other therapies, but data are limited on effectiveness. Newer promising agents include pralatrexate and CD30 antibody-drug conjugate brentuximab vedotin [89, 90]

  • Multi-agent chemotherapy is not recommended for multifocal or relapsing PC-ALCL limited to the skin; it is recommended only for extracutaneous spread

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