血吞噬细胞淋巴组织细胞增生症（HLH）是肝移植手术（LT）罕见但致命的并发症。HLH的特征是病理性巨噬细胞活化导致高细胞因子血症、过度炎症和组织破坏，进而导致器官功能进行性损害。当HLH并发风湿性疾病或自身炎症性疾病时，也称为巨噬细胞活化综合征（MAS）。测量几种血清细胞因子可能有助于诊断HLH和MAS。与巨噬细胞活化相关的细胞因子，如新型比特林、白细胞介素-18（IL-18）和可溶性肿瘤坏死因子受体（sTNF-R）I和II在HLH并发LT的患者中尚未得到评估。在该病例中，我们对LT术后围手术期对这些细胞因子进行了评估。患者是一名24岁的女性，因自身免疫性肝炎急性恶化而接受活体供肝移植手术。术后第12天，根据标准，患者被诊断为HLH。血浆置换、甲泼尼龙冲击疗法、静脉免疫球蛋白和粒细胞-集落刺激因子有效抑制了HLH的致命进展。当HLH发生在LT术后时，细胞因子分析显示新型比特林、IL-18、sTNFR-I和II升高：细胞因子风暴。值得注意的是，住院时的细胞因子分析也显示细胞因子水平升高。特别是IL-18水平显著升高，表明先天免疫系统的活化参与其中。这些结果表明，在LT前细胞因子风暴和巨噬细胞活化已经发生。基于这些发现，与巨噬细胞活化相关的细胞因子分析可能对诊断和预测LT患者的HLH和MAS有用。版权所有 © 2023 Elsevier Inc. 保留所有权利。

Hemophagocytic lymphohistiocytosis (HLH) is a rare but lethal complication of liver transplantation (LT). HLH is characterized by pathologic macrophage activation with hypercytokinemia, excessive inflammation, and tissue destruction, resulting in progressive organ dysfunction. HLH is also known as macrophage activation syndrome (MAS) when complicated by rheumatic or autoinflammatory diseases. Measuring several serum cytokines could be helpful in diagnosing HLH and MAS. Cytokines related to macrophage activation: neopterin, interleukin-18 (IL-18), and soluble tumor necrosis factor receptors (sTNF-R) I and II have not been assessed in patients with HLH complicated by LT. In this case, these cytokines were evaluated in the perioperative period of LT. The patient was a 24-year-old woman who underwent living-donor LT for acute worsening of autoimmune hepatitis. On postoperative day 12, the patient was diagnosed with HLH on the basis of the criteria. Plasma exchange, steroid pulse therapy, intravenous immunoglobulin and granulocyte-colony stimulating factor effectively inhibited progression to lethal HLH. When HLH occurred after LT, cytokine analysis showed that neopterin, IL-18, sTNFR-I, and II were elevated: cytokine storm. Of note, cytokine analysis on hospital admission also revealed elevated cytokine levels. Particularly, IL-18 levels were markedly elevated, suggesting that activation of the innate immune system was involved. These results revealed that a cytokine storm and macrophage activation developed before LT. Based on these findings, cytokine analysis related to macrophage activation may be useful for diagnosing and predicting HLH and MAS in patients with LT.Copyright © 2023 Elsevier Inc. All rights reserved.