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Blood, 15 January 2008, Vol. 111, No. 2, pp. 838-845.
Prepublished online as a Blood First Edition Paper on October 12, 2007; DOI 10.1182/blood-2007-04-087288.
 Previous Article | Next Article 
Submitted April 26, 2007
Accepted October 2, 2007
Subcutaneous panniculitis-like T-cell lymphoma: definition, classification and prognostic factors. An EORTC Cutaneous Lymphoma Group study of 83 cases
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, Jose L Rodriguez Peralto, Alistair Robson, Nancy J. Senff, Maarten H. Vermeer, Janine Wechsler, Sean Whittaker, and Chris J.L.M. Meijer
Department of Dermatology, Leiden University Medical Center, Leiden, Netherlands
Department of Pathology, Leiden University Medical Center, Leiden, Netherlands
Department of Dermatology, University of Graz, Graz, Austria
Department of Dermatology, University of MilanBiococca and Fondazione Ospedale Policlinico, Regina Elena and Mangiagalli, IRCCS, Milan, Italy
Department of Human Pathology and Oncology, University of Florence, Florence, Italy
Department of Dermatology, Skin Cancer Center, Charite-Universitatsmedizin, Berlin, Germany
Department of Pathology, University Medical Center Utrecht, Utrecht, Netherlands
Department of Dermatology, Hopital Cochin, Universite Paris 5, Paris, France
Department of Dermatology, University Hospital Gent, Gent, Belgium
Department of Dermatology, Helsinki University Hospital, Helsinki, Finland
Institute of Pathology, Campus Benjamin Franklin, Charite-Universitatsmedizin, Berlin, Germany
Department of Dermatology, University Hospital Zurich, Zurich, Switzerland
Department of Dermatology, Hospital Universitario 12 de Octubre, Madrid, Spain
Department of Pathology, University of Pavia, Pavia, Italy
Department of Pathology, University Hospital Dijon, Dijon, France
Department of Pathology, Hospital Universitario 12 de Octubre, Madrid, Spain
Skin Tumor Unit, St. John's Institute of Dermatology, St. Thomas' Hospital, London, United Kingdom
Department of Pathology, Hopital Henri Mondor, Creteil, France
Department of Pathology, Vrije Universiteit Medical Center, Amsterdam, Netherlands
* Corresponding author; email: rein.willemze{at}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 behaviour. Recent studies suggest that distinction should be made between SPTL with an  / T-cell phenotype (SPTL-AB) and SPTL with a  / T-cell phenotype (SPTL-GD), but studies are still limited. To better define their clinicopathologic features, immunophenotype, treatment, and survival, 63 SPTL-AB and 20 SPTL-GD, were studied at a workshop of the EORTC Cutaneous Lymphoma Group. SPTL-AB were generally confined to the subcutis, had a CD4-, CD8+, CD56-, F1+ phenotype, were uncommonly associated with a haemophagocytic syndrome (HPS;17%) and had a favorable prognosis (5-year OS, 82%). SPTL-AB patients without HPS had a significantly better survival than patients with HPS (5-year OS: 91% versus 46%; p < 0.001). SPTL-GD often showed (epi)dermal involvement and/or ulceration, a CD4-, CD8-, CD56+/-, F1- T-cell phenotype, and a poor prognosis (5-year OS, 11%), irrespective of the presence of HPS or the type of treatment.
These results indicate that SPTL-AB and SPTL-GD are distinct entities, and justify that the term SPTL should further only be used for SPTL-AB. SPTL-AB without associated HPS have an excellent prognosis and multi-agent chemotherapy as first choice of treatment should be questioned.
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