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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.
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NEOPLASIA
Subcutaneous panniculitis-like T-cell lymphoma: definition, classification, and prognostic factors: an EORTC Cutaneous Lymphoma Group Study of 83 cases
Rein Willemze1,
Patty M. Jansen2,
Lorenzo Cerroni3,
Emilio Berti4,
Marco Santucci5,
Chalid Assaf6,
Marijke R. Canninga-van Dijk7,
Agnes Carlotti8,
Marie-Louise Geerts9,
Sonja Hahtola10,
Michael Hummel11,
Leila Jeskanen10,
Werner Kempf12,
Cesare Massone3,
Pablo L. Ortiz-Romero13,
Marco Paulli14,
Tony Petrella15,
Annamari Ranki10,
José L. Rodriguez Peralto16,
Alistair Robson17,
Nancy J. Senff1,
Maarten H. Vermeer1,
Janine Wechsler18,
Sean Whittaker17, and
Chris J. L. M. Meijer19
1 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  /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.
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