Subcutaneous panniculitis-like T-cell lymphoma: definition, classification, and prognostic factors: an EORTC Cutaneous Lymphoma Group Study of 83 cases

Blood. 2008 Jan 15;111(2):838-45. doi: 10.1182/blood-2007-04-087288. Epub 2007 Oct 12.

Abstract

In the WHO classification, subcutaneous panniculitis-like T-cell lymphoma (SPTL) is defined as a distinct type of T-cell lymphoma with an aggressive clinical behavior. Recent studies suggest that distinction should be made between SPTL with an alpha/beta T-cell phenotype (SPTL-AB) and SPTL with a gammadelta T-cell phenotype (SPTL-GD), but studies are limited. To better define their clinicopathologic features, immunophenotype, treatment, and survival, 63 SPTL-ABs and 20 SPTL-GDs were studied at a workshop of the EORTC Cutaneous Lymphoma Group. SPTL-ABs were generally confined to the subcutis, had a CD4-, CD8+, CD56-, betaF1+ phenotype, were uncommonly associated with a hemophagocytic syndrome (HPS; 17%), and had a favorable prognosis (5-year overall survival [OS]: 82%). SPTL-AB patients without HPS had a significantly better survival than patients with HPS (5-year OS: 91% vs 46%; P<.001). SPTL-GDs often showed (epi)dermal involvement and/or ulceration, a CD4-, CD8-, CD56+/-, betaF1- T-cell phenotype, and poor prognosis (5-year OS: 11%), irrespective of the presence of HPS or type of treatment. These results indicate that SPTL-AB and SPTL-GD are distinct entities, and justify that the term SPTL should further be used only for SPTL-AB. SPTL-ABs without associated HPS have an excellent prognosis, and multiagent chemotherapy as first choice of treatment should be questioned.

Publication types

  • Multicenter Study

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • CD4 Antigens / metabolism
  • CD56 Antigen / metabolism
  • CD8 Antigens / metabolism
  • Carrier Proteins / metabolism
  • Child
  • Diagnosis, Differential
  • Disease-Free Survival
  • Education
  • Female
  • Humans
  • Lymphoma, T-Cell, Cutaneous / classification*
  • Lymphoma, T-Cell, Cutaneous / diagnosis*
  • Lymphoma, T-Cell, Cutaneous / drug therapy
  • Lymphoma, T-Cell, Cutaneous / metabolism
  • Lymphoma, T-Cell, Cutaneous / mortality*
  • Lymphoma, T-Cell, Cutaneous / pathology
  • Male
  • Microfilament Proteins / metabolism
  • Middle Aged
  • Panniculitis / classification*
  • Panniculitis / diagnosis*
  • Panniculitis / drug therapy
  • Panniculitis / metabolism
  • Panniculitis / mortality*
  • Panniculitis / pathology
  • Phenotype
  • Receptors, Antigen, T-Cell, alpha-beta / metabolism
  • Receptors, Antigen, T-Cell, gamma-delta / metabolism
  • Survival Rate
  • T-Lymphocytes / metabolism
  • T-Lymphocytes / pathology

Substances

  • CD4 Antigens
  • CD56 Antigen
  • CD8 Antigens
  • Carrier Proteins
  • Microfilament Proteins
  • Receptors, Antigen, T-Cell, alpha-beta
  • Receptors, Antigen, T-Cell, gamma-delta
  • fodrin