• Qing-Yuan Xu, Hai-Yan Yang, Mei-Wei Li, Zhen-Dong He, Hao-Yuan Hong, and Zhi-Gang Peng.
• Department of Medical Oncology, First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi Zhuang Autonomous Region, P. R. China.
• Medicine (Baltimore). 2022 Sep 23; 101 (38): e30731.

RationaleExtranodal nature killer/T-cell lymphoma (ENKTL) failing in asparaginase-containing treatments is fatal, it has a higher mortality rate when accompanied by secondary hemophagocytic lymphohistiocytosis (HLH). The study reported 2 ENKTL-related HLH patients.Patient ConcernsPatient 1 visited for nasal congestion and runny nose for 6 months then got a fever and serious myelosuppression after P-GEP (pegaspargase, gemcitabine, etoposide, and methylprednisolone) chemotherapy. Patient 2 complained of painless lymphadenectasis in the right neck for 4 months and experienced recurrent fever and poor performance status after 3 cycles of P-Gemox (pegaspargase, gemcitabine, and oxaliplatin) chemotherapy.DiagnosesPatient 1 and patient 2 were diagnosed as ENKTL failing in asparaginase-based chemotherapy and involving secondary HLH.InterventionsThe dose of chidamide was 20 mg twice a week for 2 weeks and sintilimab was 200 mg once every 3 weeks.OutcomesENKTL was relieved and the HLH was resolved after the therapy of sintilimab and chidamide. The patients had achieved durable survival without immune-related adverse events.LessonsENKTL-related HLH needs early diagnosis and treatment. The combined strategy of sintilimab plus chidamide help deal with HLH and solve ENKTL, it may be a useful treatment option for ENKTL-related HLH.Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.

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