title: Subcutaneous panniculitis-like T-cell lymphoma: definition, classification, and prognostic factors: an EORTC Cutaneous Lymphoma Group Study of 83 cases.
abstract: 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.
authors.affiliation: Department of Dermatology, Leiden University Medical Center, the Netherlands. rein.willemze@planet.nl
authors.forename: Rein
authors.initials: R
authors.lastname: Willemze
authors.forename: Patty M
authors.initials: PM
authors.lastname: Jansen
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Cerroni
authors.forename: Emilio
authors.initials: E
authors.lastname: Berti
authors.forename: Marco
authors.initials: M
authors.lastname: Santucci
authors.forename: Chalid
authors.initials: C
authors.lastname: Assaf
authors.forename: Marijke R
authors.initials: MR
authors.lastname: Canninga-van Dijk
authors.forename: Agnes
authors.initials: A
authors.lastname: Carlotti
authors.forename: Marie-Louise
authors.initials: ML
authors.lastname: Geerts
authors.forename: Sonja
authors.initials: S
authors.lastname: Hahtola
authors.forename: Michael
authors.initials: M
authors.lastname: Hummel
authors.forename: Leila
authors.initials: L
authors.lastname: Jeskanen
authors.forename: Werner
authors.initials: W
authors.lastname: Kempf
authors.forename: Cesare
authors.initials: C
authors.lastname: Massone
authors.forename: Pablo L
authors.initials: PL
authors.lastname: Ortiz-Romero
authors.forename: Marco
authors.initials: M
authors.lastname: Paulli
authors.forename: Tony
authors.initials: T
authors.lastname: Petrella
authors.forename: Annamari
authors.initials: A
authors.lastname: Ranki
authors.forename: José L Rodriguez
authors.initials: JL
authors.lastname: Peralto
authors.forename: Alistair
authors.initials: A
authors.lastname: Robson
authors.forename: Nancy J
authors.initials: NJ
authors.lastname: Senff
authors.forename: Maarten H
authors.initials: MH
authors.lastname: Vermeer
authors.forename: Janine
authors.initials: J
authors.lastname: Wechsler
authors.forename: Sean
authors.initials: S
authors.lastname: Whittaker
authors.forename: Chris J L M
authors.initials: CJ
authors.lastname: Meijer
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D015704:CD4 Antigens / Q000378:metabolism; D019002:CD56 Antigen / Q000378:metabolism; D016827:CD8 Antigens / Q000378:metabolism; D002352:Carrier Proteins / Q000378:metabolism; D002648:Child; D003937:Diagnosis, Differential; D018572:Disease-Free Survival; D004493:Education; D005260:Female; D006801:Humans; D016410:Lymphoma, T-Cell, Cutaneous / Q000145:classification* / Q000175:diagnosis* / Q000188:drug therapy / Q000378:metabolism / Q000401:mortality* / Q000473:pathology; D008297:Male; D008840:Microfilament Proteins / Q000378:metabolism; D008875:Middle Aged; D015434:Panniculitis / Q000145:classification* / Q000175:diagnosis* / Q000188:drug therapy / Q000378:metabolism / Q000401:mortality* / Q000473:pathology; D010641:Phenotype; D016693:Receptors, Antigen, T-Cell, alpha-beta / Q000378:metabolism; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000378:metabolism; D015996:Survival Rate; D013601:T-Lymphocytes / Q000378:metabolism / Q000473:pathology; D013049:Spectrin
chemical_list: D015704:CD4 Antigens; D019002:CD56 Antigen; D016827:CD8 Antigens; D002352:Carrier Proteins; D008840:Microfilament Proteins; D016693:Receptors, Antigen, T-Cell, alpha-beta; D016692:Receptors, Antigen, T-Cell, gamma-delta; D013049:Spectrin