Subcutaneous panniculitis-like T-cell lymphoma: definition, classification, and prognostic factors: an EORTC Cutaneous Lymphoma Group Study of 83 cases.
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.
Willemze
Rein
R
Department of Dermatology, Leiden University Medical Center, the Netherlands. rein.willemze@planet.nl
Jansen
Patty M
PM
Cerroni
Lorenzo
L
Berti
Emilio
E
Santucci
Marco
M
Assaf
Chalid
C
Canninga-van Dijk
Marijke R
MR
Carlotti
Agnes
A
Geerts
Marie-Louise
ML
Hahtola
Sonja
S
Hummel
Michael
M
Jeskanen
Leila
L
Kempf
Werner
W
Massone
Cesare
C
Ortiz-Romero
Pablo L
PL
Paulli
Marco
M
Petrella
Tony
T
Ranki
Annamari
A
Peralto
José L Rodriguez
JL
Robson
Alistair
A
Senff
Nancy J
NJ
Vermeer
Maarten H
MH
Wechsler
Janine
J
Whittaker
Sean
S
Meijer
Chris J L M
CJ
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
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