CD30+ cutaneous lymphoproliferative disorders: the Stanford experience in lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma

J Am Acad Dermatol. 2003 Dec;49(6):1049-58. doi: 10.1016/s0190-9622(03)02484-8.

Abstract

Background: CD30+ cutaneous lymphoproliferative disorders (CLPDs) include lymphomatoid papulosis, borderline cases of CD30+CLPDs, and primary cutaneous anaplastic large cell lymphoma (PCALCL). Prior studies have shown CD30+CLPDs have an excellent prognosis.

Objective: We sought to present the single-center experience of Stanford University, Stanford, Calif, in the management of CD30+CLPDs.

Methods: A retrospective cohort analysis of 56 patients with CD30+CLPDs treated at our institution was performed.

Results: No patients with lymphomatoid papulosis died of disease, and overall survival was 92% at 5 and 10 years. Disease-specific survivals at 5 and 10 years for PCALCL were 85%. Disease-specific survival at 5 years for localized versus generalized PCALCL was 91% versus 50% (P =.31). PCALCL was highly responsive to treatment, but the relapse rate was 42%. In all, 3 patients progressed to extracutaneous stage of disease. No clinical or histologic factors analyzed were predictive of worse outcome in lymphomatoid papulosis and PCALCL.

Conclusion: Similar to prior reports from multicenter European groups, the single-center experience at our institution demonstrates CD30+CLPDs have an overall excellent prognosis; however, cases of PCALCL with poor outcome do exist.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Cohort Studies
  • Humans
  • Immunohistochemistry
  • Ki-1 Antigen / blood*
  • Lymphoma, Large-Cell, Anaplastic / immunology
  • Lymphoma, Large-Cell, Anaplastic / mortality
  • Lymphoma, Large-Cell, Anaplastic / pathology*
  • Lymphoma, Large-Cell, Anaplastic / therapy
  • Lymphomatoid Papulosis / immunology
  • Lymphomatoid Papulosis / mortality
  • Lymphomatoid Papulosis / pathology*
  • Lymphomatoid Papulosis / therapy
  • Prognosis
  • Retrospective Studies
  • Skin Neoplasms / immunology
  • Skin Neoplasms / mortality
  • Skin Neoplasms / pathology*
  • Skin Neoplasms / therapy
  • Survival Rate

Substances

  • Ki-1 Antigen