Prognostic Factors in Primary Cutaneous Lymphomas Other than Mycosis Fungoides and the Sézary Syndrome

Author: F. Grange, G. Hedelin, P. Joly, et al.
Content Contributor: Abramson Cancer Center of the University of Pennsylvania
Last Reviewed: November 01, 2001

Reviewers: Li Liu, MD
Source: Blood, 93(11), June 1999


Data on prognostic factors of primary cutaneous lymphomas (PCL) other than mycosis fungoides (MF) and the S?zary Syndrome (SS) has been lacking. The well-established factors associated with poor prognosis for nodal lymphomas may not hold true for PCL. Grange and associates conducted this study to test the clinical validity of the EORTC classification and to identify important prognostic factors of survival in patients with non-MF/SS PCL.


A retrospective review of one hundred and fifty-eight patients, median age 68, with diagnoses of non-MF/SS PCL was performed. PCL was defined by the absence of extracutaneous disease detected at the time of diagnosis. Univariate and multivariate analyses for many possible prognostic factors were performed.

  • The median relative survival time was 81 months and 5-year overall relative survival rate was 73%.
  • The most important prognostic factors were clinical classification (EORTC prognostic group) and generalized distribution of skin lesions, especially tumor involvement of more than one anatomic site.
  • Age, presence of B symptoms, and a high LDH level were not found to be significant prognostic factors on multivariate analysis.

Optimal treatments of non-MF/SS PCL remain undefined. Radiation therapy alone can be used in patients with localized CD30 positive large T-cell lymphomas and localized indolent B-cell lymphomas. Multi-agent chemotherapy is more suitable for patients with more advanced disease.

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