Subcutaneous panniculitis-like T-cell lymphoma: definition, classification, and prognostic factors: an EORTC Cutaneous Lymphoma Group Study of 83 cases.
Willemze R,
Jansen PM,
Cerroni L,
Berti E,
Santucci M,
Assaf C,
Canninga-van Dijk MR,
Carlotti A,
Geerts ML,
Hahtola S,
Hummel M,
Jeskanen L,
Kempf W,
Massone C,
Ortiz-Romero PL,
Paulli M,
Petrella T,
Ranki A,
Peralto JL,
Robson A,
Senff NJ,
Vermeer MH,
Wechsler J,
Whittaker S,
Meijer CJ;
EORTC Cutaneous Lymphoma Group.
Department of Dermatology, Leiden University Medical Center, the Netherlands. rein.willemze@planet.nl
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.
PMID: 17934071 [PubMed - indexed for MEDLINE]