Severe acute liver injury (ALI) is mostly triggered by viral infections and hepatotoxic drugs; however, it can also be seen in systemic diseases. Hemophagocytic lymphohistiocytosis (HLH) is a rare, immune-mediated syndrome that presents as a life-threatening inflammatory disorder affecting multiple organs. Secondary causes occur mainly in the set of malignancy, infection, and autoimmune disease, and are seldom triggered by vaccination. Although liver involvement is common, presentation as severe ALI is rare. We describe a case of a 65-year-old male with history of low-risk chronic lymphocytic leukemia and rheumatoid arthritis treated with prednisolone who presented with persistent fever and jaundice 1 week after COVID-19 vaccination. The diagnosis was challenging given the predominant liver impairment, characterized by hyperbilirubinemia, transaminases over 1,000 U/L, and prolonged INR, which prompted an extensive investigation and exclusion of autoimmune, toxic, and viral causes of hepatitis. Laboratory workup revealed bicytopenia, hyperferritinemia, which together with organ failure and evidence of hemophagocytosis in bone marrow suggested the diagnosis of HLH. After excluding infectious etiologies, flare of rheumatological disease, and the progression of hematological disease, HLH was diagnosed. He was successfully treated with etoposide and corticosteroids, with dramatic improvement of liver tests. After exclusion of other causes of secondary HLH, the recent vaccination for COVID-19 was the likely trigger. We report a case of double rarity of HLH, as it presented with severe liver dysfunction which was probably triggered by vaccination. In this case, the predominant liver involvement urged extensive investigation of liver disease, so a high index of suspicion was required to make an early diagnosis. Clinicians should consider HLH in patients with unexplained signs and symptoms of systemic inflammatory response and multiorgan involvement, including severe liver involvement as the first presentation.
GE Port J Gastroenterol

中文翻译：

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继发性噬血细胞性淋巴组织细胞增多症所致严重急性肝损伤：一例报告

严重急性肝损伤（ALI）多由病毒感染和肝毒性药物引发；然而，它也可见于全身性疾病。噬血细胞性淋巴组织细胞增多症 (HLH) 是一种罕见的免疫介导综合征，表现为一种影响多个器官的危及生命的炎症性疾病。次要原因主要发生在恶性肿瘤、感染和自身免疫性疾病中，很少由疫苗接种引发。尽管肝脏受累很常见，但表现为严重 ALI 的情况很少见。我们描述了一名 65 岁男性病例，他有低危慢性淋巴细胞白血病和类风湿性关节炎病史，接受泼尼松龙治疗，在接种 COVID-19 疫苗 1 周后出现持续发烧和黄疸。鉴于以高胆红素血症、转氨酶超过 1,000 U/L 和 INR 延长为特征的主要肝功能损害，诊断具有挑战性，这促使进行广泛的调查并排除肝炎的自身免疫、毒性和病毒原因。实验室检查显示双细胞减少、高铁蛋白血症，再加上器官衰竭和骨髓中噬血细胞增多的证据，提示 HLH 的诊断。在排除感染性病因、风湿病发作和血液病进展后，诊断为 HLH。他成功接受了依托泊苷和皮质类固醇的治疗，肝脏检查结果显着改善。在排除继发性 HLH 的其他原因后，最近接种的 COVID-19 疫苗可能是触发因素。我们报告了一例双重罕见的 HLH 病例，因为它表现出严重的肝功能障碍，这可能是由疫苗接种引发的。在这种情况下，主要的肝脏受累需要对肝脏疾病进行广泛的检查，因此需要高度怀疑才能做出早期诊断。对于出现无法解释的全身炎症反应体征和症状以及多器官受累（包括以严重肝脏受累为首发表现）的患者，临床医生应考虑 HLH。
GE Port J 胃肠科