THE DIVERSITY OF PRESENTATION AND MANAGEMENT OF SUBCUTANEOUS PANNICULITIS –LIKE T-CELL LYMPHOMA WITH ASSOCIATED HEMOPHAGOCYTIC SYNDROME - CASE SERIES ANALYSIS

• Published in: Hematology, Transfusion and Cell Therapy Vol. 44; p. S32
• Main Authors: Giza, Agnieszka, Jedras, Justyna, Rozploch, Maria, Czepko, Ewa, Jonca, Marcin, Zimowska-Curylo, Dagmara, Wilk, Mateusz, Razny, Malgorzata, Balwierz, Walentyna, Sacha, Tomasz
• Format: Journal Article
• Language: English
• Published: Elsevier 01.10.2022
• ISSN: 2531-1379
• DOI: 10.1016/j.htct.2022.09.1246

Objective: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare hematological malignancy affecting subcutaneous adipose tissue, typically with no involvement of the lymph nodes. SPTCL is associated with the increased risk of the hemophagocytic syndrome (HPS), significantly affecting prognosis and overall survival. This study aimed to present different clinical characteristics, management strategies, and outcomes in three patients diagnosed with SPTCL. Methodology: A retrospective study of the three patients diagnosed with SPTCL admitted to Hematology Departments in Krakow was conducted. Collected data included patients’ clinical characteristics and symptoms, laboratory testing, imaging tests, implemented treatment strategies and response assessment. Results: The analyzed patients (aged 15-35), presented lesions involving mainly skin in 2 patients, and mesenterium in one subject; HPS was confirmed in each case. The first line treatment consisted of HLH protocols followed by next line chemotherapies in two patients, and then with high dose therapy in one case. Cyclosporine A (CyA) was implemented in two patients, and in one case this was an initial choice. CR was achieved in 2 patients, including the subject treated with CyA from the beginning. Conclusion: This series shows a diversity of presentations and implemented management in three patients. Since SPTCL is an extremely rare condition with no standardized established therapy, choosing the optimal treatment approach is a relevant problem. The increasing data shows the effectiveness and safety of immunosuppressive treatment with CyA versus intensive chemotherapy and supports the application of CyA also in patients with developed HPS.