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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.
NEOPLASIA Subcutaneous panniculitis-like T-cell lymphoma: definition, classification, and prognostic factors: an EORTC Cutaneous Lymphoma Group Study of 83 cases1 Department of Dermatology and 2 Department of Pathology, Leiden University Medical Center, Leiden, the Netherlands; 3 Department of Dermatology, University of Graz, Graz, Austria; 4 Department of Dermatology, University of Milan-Biococca and Fondazione Ospedale Policlinico, Regina Elena and Mangiagalli, Istituti di ricovero e cura a carattere scientifico (IRCCS), Milan, Italy; 5 Department of Human Pathology and Oncology, University of Florence, Florence, Italy; 6 Department of Dermatology, Skin Cancer Center, Charité-Universitätsmedizin, Berlin, Germany; 7 Department of Pathology, University Medical Center Utrecht, Utrecht, the Netherlands; 8 Department of Dermatology, Hôpital Cochin, Université Paris 5, Paris, France; 9 Department of Dermatology, University Hospital Gent, Gent, Belgium; 10 Department of Dermatology, Helsinki University Hospital, Helsinki, Finland; 11 Institute of Pathology, Campus Benjamin Franklin, Charité Universitätsmedizin, Berlin, Germany; 12 Department of Dermatology, University Hospital Zürich, Zürich, Switzerland; 13 Department of Dermatology, Hospital Universitario 12 de Octubre, Madrid, Spain; 14 Department of Pathology, University of Pavia, Pavia, Italy; 15 Department of Pathology, University Hospital Dijon, Dijon, France; 16 Department of Pathology, Hospital Universitario 12 de Octubre, Madrid, Spain; 17 Skin Tumour Unit, St John's Institute of Dermatology, St Thomas' Hospital, London, United Kingdom; 18 Department of Pathology, Hôpital Henri Mondor, Creteil, France; and 19 Department of Pathology, Vrije Universiteit Medical Center, Amsterdam, the Netherlands
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
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| Copyright © 2008 by American Society of Hematology Online ISSN: 1528-0020 | |||||||||
/β T-cell phenotype (SPTL-AB) and SPTL with a 
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–, βF1+ 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+/–, βF1– 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.