• Lingling Xu, Xianqi Liu, Yan Wang, Yanming Wang, Xiaoxia Chu, and Liming Chen.
• Department of Hematology, Yantai Yuhuangding Hospital Affilliated to Qingdao University, Yantai, China.
• Medicine (Baltimore). 2022 Mar 4; 101 (9): e28943e28943.

RationaleHemophagocytic syndrome (HPS) is associated with a high mortality rate, and Epstein-Barr virus infection and hematological malignancies, especially T/natural killer cell lymphomas, are the most common causes; however, due to the complexity of clinical manifestations, the diagnosis is usually delayed. There are few reports of lymphoma-associated HPS (LAPS) in combination with bone marrow necrosis, and there is still no standard treatment for LAPS.Patient ConcernsA 64-year-old man developed a fever, mild jaundice, fatigue, and bone pain. Positron emission tomography and bone marrow biopsy with immunohistochemistry were performed.DiagnosisImaging analysis and bone marrow examinations were compatible with HPS, T-cell lymphoma, and bone marrow necrosis.InterventionsThe patient received combination therapy of rituximab and Cyclophosphamide, epirubicin, vincristine, glucocorticoid, etoposide.OutcomesThe patient achieved complete remission and a disease-free survival of 52 months.LessonsHPS and its potential diseases should be diagnosed and treated as soon as possible. Clinicians should be aware of the presence of lymphoma in patients with HPS. Rituximab plays an important role in the prognosis of HPS, particularly Epstein-Barr virus positivity. Cyclophosphamide, epirubicin, vincristine, glucocorticoid remains an effective regimen for the treatment of T-cell LAPS. This study provides a better understanding of the diagnosis and treatment of LAPS.Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.

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