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Hemophagocytic lymphohistiocytosis (HLH) is a rare hematologic syndrome characterized by massive, uncontrolled cytokine release, which can lead to multiple organ failure and is associated with a high mortality rate. Recent studies have found that checkpoint inhibitors (ICIs) can induce HLH. This case report describes a 75-year-old man with metastatic esophageal squamous cell carcinoma (ESCC) who developed sintilimab-associated HLH, presenting as refractory fever and respiratory symptoms initially misdiagnosed as infection. The diagnosis, confirmed via elevated ferritin, sCD25, and reduced NK-cell activity, was delayed due to nonspecific symptoms. Following the initiation of dexamethasone, the patient’s symptoms resolved and laboratory parameters normalized, leading to complete remission. The case highlights HLH as a rare but life-threatening immune-related adverse event of checkpoint inhibitors, underscores the importance of early multidisciplinary discussion (MDT) in patients with unexplained fever, and demonstrates the early diagnosis and treatment are critical.
Zhi et al. (Wed,) studied this question.