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Selected Publication:
SHR Neuro Cancer Cardio Lipid Metab Microb
Willemze, R; Jansen, PM; Cerroni, L; Berti, E; Santucci, M; Assaf, C; Dijk, MRCV; Carlotti, A; Geerts, ML; Hahtola, S; Hummel, M; Jeskanen, L; Kempf, W; Massone, C; Ortiz-Romero, PL; Paulli, M; Petrella, T; Ranki, A; Peralto, JLR; Robson, A; Senff, NJ; Vermeer, MH; Wechsler, J; Whittaker, S; Meijer, CJLM.
Subcutaneous panniculitis-like T-cell lymphoma: definition, classification, and prognostic factors: an EORTC Cutaneous Lymphoma Group Study of 83 cases.
BLOOD. 2008; 111(2): 838-845.
Doi: 10.1182/blood-2007-04-087288
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- Co-authors Med Uni Graz
- Cerroni Lorenzo
- Massone Cesare
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- 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.
- Find related publications in this database (using NLM MeSH Indexing)
- Adolescent -
- Adult -
- Aged -
- Antigens, CD4 - metabolism
- Antigens, CD56 - metabolism
- Antigens, CD8 - metabolism
- Antineoplastic Combined Chemotherapy Protocols - therapeutic use
- Carrier Proteins - metabolism
- Child -
- Diagnosis, Differential -
- Disease-Free Survival -
- Education -
- Female -
- Humans -
- Lymphoma, T-Cell, Cutaneous - classification Lymphoma, T-Cell, Cutaneous - diagnosis Lymphoma, T-Cell, Cutaneous - drug therapy Lymphoma, T-Cell, Cutaneous - metabolism Lymphoma, T-Cell, Cutaneous - mortality Lymphoma, T-Cell, Cutaneous - pathology
- Male -
- Microfilament Proteins - metabolism
- Middle Aged -
- Panniculitis - classification Panniculitis - diagnosis Panniculitis - drug therapy Panniculitis - metabolism Panniculitis - mortality Panniculitis - pathology
- Phenotype -
- Receptors, Antigen, T-Cell, alpha-beta - metabolism
- Receptors, Antigen, T-Cell, gamma-delta - metabolism
- Survival Rate -
- T-Lymphocytes - metabolism T-Lymphocytes - pathology