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

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.

• Authors: Rein Willemze, Patty M Jansen, Lorenzo Cerroni, Emilio Berti, Marco Santucci, Chalid Assaf, Marijke R Canninga-van Dijk, Agnes Carlotti, Marie-Louise Geerts, Sonja Hahtola, Michael Hummel, Leila Jeskanen, Werner Kempf, Cesare Massone, Pablo L Ortiz-Romero, Marco Paulli, Tony Petrella, Annamari Ranki, José L Rodriguez Peralto, Alistair Robson, Nancy J Senff, Maarten H Vermeer, Janine Wechsler, Sean Whittaker, Chris J L M Meijer, EORTC Cutaneous Lymphoma Group
• Journal: Blood (Blood) Vol. 111 Issue 2 Pg. 838-45 (Jan 15 2008) ISSN: 0006-4971 [Print] United States
• PMID: 17934071 (Publication Type: Journal Article, Multicenter Study)

Chemical References

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

Topics

• 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, diagnosis, drug therapy, metabolism, mortality, pathology)
• Male
• Microfilament Proteins (metabolism)
• Middle Aged
• Panniculitis (classification, diagnosis, drug therapy, metabolism, mortality, pathology)
• Phenotype
• Receptors, Antigen, T-Cell, alpha-beta (metabolism)
• Receptors, Antigen, T-Cell, gamma-delta (metabolism)
• Survival Rate
• T-Lymphocytes (metabolism, pathology)