Goodman Alexander C, Burns Austin J, Tullberg Ian
Medicine, Rocky Vista University College of Osteopathic Medicine, Parker, USA.
Family Medicine, University of Colorado Health Memoral Hospital System, Colorado Springs, USA.
Cureus. 2025 May 8;17(5):e83720. doi: 10.7759/cureus.83720. eCollection 2025 May.
Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory disorder associated with infections, malignancies, autoimmune conditions, and inflammatory states. HLH is characterized by unregulated cytokine release and immune activation, leading to widespread tissue damage. In this case, we describe a male in his 30s who presented to the emergency department with concerns about sepsis after being diagnosed with mastoiditis a week earlier. Further investigation revealed persistent fevers, pancytopenia, hepatosplenomegaly, hyperferritinemia, and hypertriglyceridemia, fulfilling the diagnostic criteria for HLH. Additional serologic testing revealed a significantly elevated Epstein-Barr virus (EBV) viral load on PCR, with positive immunoglobulin G (IgG) and negative IgM, consistent with EBV reactivation. HLH in the setting of EBV reactivation is rare and may be associated with a poor prognosis. Central pontine myelinolysis is a commonly fatal neurologic condition that is rarely attributed to HLH but should be considered when other causes are ruled out such as electrolyte derangements and rapid correction.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见的高炎症性疾病，与感染、恶性肿瘤、自身免疫性疾病及炎症状态相关。HLH的特征是细胞因子释放失控和免疫激活，导致广泛的组织损伤。在此病例中，我们描述了一名30多岁男性，他在一周前被诊断为乳突炎后因担心败血症而前往急诊科就诊。进一步检查发现持续发热、全血细胞减少、肝脾肿大、高铁蛋白血症和高甘油三酯血症，符合HLH的诊断标准。额外的血清学检测显示，PCR检测的爱泼斯坦-巴尔病毒（EBV）病毒载量显著升高，免疫球蛋白G（IgG）阳性而IgM阴性，符合EBV再激活。EBV再激活情况下的HLH很罕见，且可能预后不良。桥脑中央髓鞘溶解症是一种通常致命的神经系统疾病，很少归因于HLH，但在排除其他原因（如电解质紊乱和快速纠正）时应予以考虑。
