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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. This Article Full Text (PDF) All Versions of this Article: blood-2007-04-087288v1 111/2/838    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Willemze, R. Articles by Meijer, C. J.L.M. Search for Related Content PubMed PubMed Citation Articles by Willemze, R. Articles by Meijer, C. J.L.M. Social Bookmarking                   What's this?  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. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

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