Subcutaneous panniculitis-like T-cell lymphoma: Clinical features, therapeutic approach, and outcome in a case series of 16 patients
| Autor: | Isabel Bielsa, Pedro Sánchez-Sambucety, Teresa Estrach, Juan I. Yanguas-Bayona, Yeray Peñate, Pablo L. Ortiz-Romero, Rosa M. Martí, Virginia Velasco-Tamariz, Josune Mitxelena, Vicente García-Patos, Ingrid Lopez-Lerma, Fernando Gallardo, Ramon M. Pujol |
|---|---|
| Rok vydání: | 2018 |
| Předmět: |
Adult
Male medicine.medical_specialty Panniculitis Skin Neoplasms Dermatology CHOP Lymphoma T-Cell Risk Assessment Gastroenterology Disease-Free Survival Sampling Studies Cohort Studies Young Adult 030207 dermatology & venereal diseases 03 medical and health sciences 0302 clinical medicine Subcutaneous Panniculitis-Like T-Cell Lymphoma Positron Emission Tomography Computed Tomography Internal medicine medicine Humans Survival rate Aged Retrospective Studies subcutaneous panniculitis-like T-cell lymphoma treatment business.industry cutaneous Retrospective cohort study Chemoradiotherapy Middle Aged medicine.disease Survival Analysis Lymphoma T-Cell Cutaneous Lymphoma Spain 030220 oncology & carcinogenesis Female Methotrexate Histopathology business CD8 medicine.drug |
| Zdroj: | Journal of the American Academy of Dermatology r-IGTP. Repositorio Institucional de Producción Científica del Instituto de Investigación Germans Trias i Pujol instname |
| ISSN: | 0190-9622 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|
Full Text from ScienceDirect