Department of Internal Medicine, Tokyo Bay Urayasu Ichikawa Medical Center, Urayasu, Chiba, Japan.
Department of Rheumatology, Tokyo Bay Urayasu Ichikawa Medical Center, Urayasu, Chiba, Japan.
Am J Case Rep. 2021 Oct 17;22:e933272. doi: 10.12659/AJCR.933272.
BACKGROUND Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening systemic hyperinflammatory condition. Most adult HLH cases are secondary to infection, malignancy, and rheumatic diseases. Epstein-Barr virus (EBV) infection is the most frequent cause of infection-induced HLH. Early treatment with dexamethasone, etoposide, and cyclosporine is generally recommended for adult patients with HLH. However, this treatment regimen was established based on pediatric clinical trial data; thus, its efficacy and validity in adults remain unclear. Because little is known about the disease course of untreated adult EBV-associated HLH (EBV-HLH), we report a case of an adult patient who recovered from EBV-HLH spontaneously without specific treatment and discuss potential treatment strategies. CASE REPORT A 34-year-old man presented to the emergency department with a 7-day history of fever, headache, and sore throat. The main laboratory test abnormalities were elevated liver enzymes, hyperbilirubinemia, hypertriglyceridemia, and hyperferritinemia. Serologic tests confirmed acute primary EBV infection. He was diagnosed with EBV-HLH based on the HLH-2004 diagnostic criteria and the HLH probability calculator (HScore). Because he was clinically stable, we did not initiate immunosuppressive/cytotoxic treatment targeting HLH. High-grade fever persisted, but the abnormalities in his laboratory data improved spontaneously, and he did not develop major organ failure. His fever resolved on day 29 without HLH-specific treatment. CONCLUSIONS In clinically stable adult patients with EBV-HLH without major organ failure, it might be an acceptable alternative to observe the patient for several weeks before initiating HLH-specific treatment. Further research is required to better predict the subset of patients who can be safely observed without treatment.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见的危及生命的全身性炎症反应过度综合征。大多数成人 HLH 病例继发于感染、恶性肿瘤和风湿性疾病。EB 病毒（EBV）感染是感染相关性 HLH 最常见的病因。一般建议对成人 HLH 患者早期采用地塞米松、依托泊苷和环孢素治疗。然而，该治疗方案是基于儿科临床试验数据建立的；因此，其在成人中的疗效和有效性尚不清楚。由于成人未经治疗的 EBV 相关性 HLH（EBV-HLH）的疾病过程知之甚少，我们报告了一例成人患者在未接受特定治疗的情况下自发从 EBV-HLH 中康复，并讨论了潜在的治疗策略。
一名 34 岁男性因发热、头痛和咽痛 7 天就诊于急诊。主要实验室检查异常包括肝酶升高、高胆红素血症、高甘油三酯血症和高铁蛋白血症。血清学检查证实为急性原发性 EBV 感染。根据 HLH-2004 诊断标准和 HLH 概率计算器（HScore），他被诊断为 EBV-HLH。由于他临床稳定，我们没有启动针对 HLH 的免疫抑制/细胞毒性治疗。高热持续存在，但实验室数据异常自发改善，他没有发生主要器官衰竭。他的发热在第 29 天自行消退，没有接受 HLH 特异性治疗。
在没有主要器官衰竭的临床稳定的 EBV-HLH 成人患者中，在启动 HLH 特异性治疗前观察患者数周可能是一种可以接受的替代方案。需要进一步研究以更好地预测可以安全观察而无需治疗的患者亚组。
