“…• Prognosis and treatment: It has an unfavorable prognosis with a median survival time of 12-15 months (Mraz-Gernhard et al 2001). Chemotherapy, including SMILE regime (dexamethasone, methotrexate, ifosfamide, L-asparaginase, and etoposide), alone or in association with radiotherapy, has been confirmed to improve patient outcomes (Guo et al 2016). • Differential diagnosis: Other CD56 + and/or EBV-associated T or NK cell lymphomas, primary cutaneous γ/δ T cell lymphoma, subcutaneous panniculitislike T cell lymphoma (Kempf and Mitteldorf 2015), leprosy, leishmaniasis, syphilis, rhinoscleroma, and Wegener disease (Schuler et al 2017) 2.15 Angioimmunoblastic T Cell Lymphoma Zinzani 2017).…”