Subcutaneous panniculitis-like T-cell lymphoma: Clinical features, therapeutic approach, and outcome in a case series of 16 patients
Por:
Lopez-Lerma, I, Penate, Y, Gallardo, F, Marti, RM, Mitxelena, J, Bielsa, I, Velasco-Tamariz, V, Yanguas-Bayona, JI, Sanchez-Sambucety, P, Garcia-Patos, V, Ortiz-Romero, PL, Pujol, RM and Estrach, T
Publicada:
1 nov 2018
Categoría:
Dermatology
Resumen:
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.
Filiaciones:
Lopez-Lerma, I:
Hosp Univ Vall dHebron, Barcelona, Spain
Penate, Y:
Complejo Hosp Univ Insular Maternoinfantil Gran C, Las Palmas Gran Canaria, Spain
Gallardo, F:
Hosp Mar, Barcelona, Spain
Marti, RM:
Hosp Arnau Vilanova, Lleida, Spain
Mitxelena, J:
Complejo Hosp Navarra, Pamplona, Spain
:
Hosp Univ Germans Trias i Pujol, Badalona, Spain
Velasco-Tamariz, V:
Hosp Univ 12 Octubre, Madrid, Spain
Yanguas-Bayona, JI:
Complejo Hosp Navarra, Pamplona, Spain
Sanchez-Sambucety, P:
Complejo Asistencial Univ Leon, Leon, Spain
Garcia-Patos, V:
Hosp Univ Vall dHebron, Barcelona, Spain
Ortiz-Romero, PL:
Hosp Univ 12 Octubre, Madrid, Spain
Pujol, RM:
Hosp Mar, Barcelona, Spain
Estrach, T:
Hosp Clin, Leon, Spain
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