Subcutaneous panniculitis-like T-cell lymphoma: Clinical features, therapeutic approach, and outcome in a case series of 16 patients - 15/10/18
, Yeray Peñate, MD, PhD b, Fernando Gallardo, MD, PhD c, Rosa M. Martí, MD, PhD d, Josune Mitxelena, MD e, Isabel Bielsa, MD, PhD f, Virginia Velasco-Tamariz, MD g, Juan I. Yanguas-Bayona, MD e, Pedro Sánchez-Sambucety, MD h, Vicente García-Patos, MD, PhD a, Pablo L. Ortiz-Romero, MD, PhD g, Ramón M. Pujol, MD, PhD c, Teresa Estrach, MD, PhD iAbstract |
Background |
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare primary cutaneous lymphoma of mature cytotoxic T cells. Initially, patients with SPTCL were treated with doxorubicin-based polychemotherapy.
Objective |
To analyze clinical, biologic, immunophenotypical, molecular, imaging, treatment, and outcome data reflecting the current state of knowledge.
Methods |
A retrospective multicenter study of 16 patients with SPTCL that was diagnosed between 1996 and 2016.
Results |
The female-to-male ratio was 1.7. The median age at diagnosis was 46.5 years. Patients presented with multiple nodular or plaque-like lesions preferentially affecting the legs and/or trunk. Histopathology typically showed a lobular panniculitis with individual adipocytes surrounded by atypical lymphocytes, usually with a CD3+, CD4–, CD8+, CD56–, TIA1 cytotoxic granule associated RNA binding protein 1–positive phenotype and high proliferation rate. SPTCL was associated with autoimmune diseases in 25% of patients, and with the development of hemophagocytic syndrome in 18% of patients. Oral steroids alone or in combination with low-dose methotrexate or cyclosporine A were the most common initial treatment, achieving a complete response in 85% of the treated patients. The median follow-up time was 14 months. The 5-year disease-specific survival rate was 85.7%.
Limitations |
This was a retrospective study.
Conclusions |
SPTCL has an excellent prognosis. Immunosuppressive agents can be considered for first-line treatment.
Il testo completo di questo articolo è disponibile in PDF.Key words : cutaneous, subcutaneous panniculitis-like T-cell lymphoma, treatment
Abbreviations used : CHOP, EORTC, FDG, HPS, PET/CT, SPTCL
Mappa
| Funding sources: None. |
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| Conflicts of interest: None disclosed. |
Vol 79 - N° 5
P. 892-898 - novembre 2018 Ritorno al numero
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