Pesic, Andrej (59180851600)Andrej (59180851600)PesicVukovic, Vojin (56180315400)Vojin (56180315400)VukovicKozarac, Sofija (58973969700)Sofija (58973969700)KozaracOtasevic, Vladimir (57219923471)Vladimir (57219923471)OtasevicBibic, Tamara (59180992200)Tamara (59180992200)BibicMihaljevic, Biljana (6701325767)Biljana (6701325767)MihaljevicAntic, Darko (23979576100)Darko (23979576100)Antic2025-07-022025-07-022024https://doi.org/10.5937/jomb0-48290https://www.scopus.com/inward/record.uri?eid=2-s2.0-85196536458&doi=10.5937%2fjomb0-48290&partnerID=40&md5=87e8e4c6cf0d995b7f30812a4d5e4f5chttps://remedy.med.bg.ac.rs/handle/123456789/11758Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hyperinflammatory disorder characterized by dysfunction of NK cells and cytotoxic lymphocytes. We present a rare case of a patient diagnosed with HLH who presented with persistent fever during treatment for refractory T-cell/histiocyte-rich large B-cell lymphoma (TCHRLBCL), highlighting the challenges of managing HLH in the context of refractory lymphoma. According to our review of the literature, this is the first case of HLH that developed several months into treatment for refractory TCHRLBCL and not in close temporal relation to lymphoma diagnosis. © 2024 Society of Medical Biochemists of Serbia. All rights reserved.case reporthemophagocytic lymphohistiocytosisimmune-related adverse eventrefractory lymphomareview of the literatureT-cell/histiocyte-rich large B cell lymphoma