Quadri Syed P, Jain Nitesh K, Brandon Brooke L, Modi Harshit, Bawaadam Hasnain
Medicine, Aurora Medical Center, Kenosha, USA.
Critical Care Medicine, Mayo Clinic, Mankato, USA.
Cureus. 2020 Aug 5;12(8):e9561. doi: 10.7759/cureus.9561.
Hemophagocytic lymphohistiocytosis (HLH) is an immune related clinical syndrome with protean manifestations, varying presentation, clinically complex, with diverse causes, and is an under-recognized entity which carries high morbidity and mortality. It is precipitated by an immunological trigger in a susceptible host resulting in immune activation and dysregulation leading to disruption of immune homeostasis, cytokine storm and multi-organ failure. We describe a case of Epstein-Barr virus (EBV) associated HLH with its typical diagnostic challenges and associated high mortality rate. Certain diagnostic criteria and online tools may help to arrive at an earlier presumptive diagnosis which, in turn, may expedite treatment and lead to better clinical outcomes.
噬血细胞性淋巴组织细胞增生症（HLH）是一种与免疫相关的临床综合征，表现形式多样，临床表现复杂，病因各异，是一种认识不足但发病率和死亡率高的疾病。它由易感宿主中的免疫触发因素引发，导致免疫激活和失调，进而破坏免疫稳态、引发细胞因子风暴和多器官功能衰竭。我们描述了一例与 Epstein-Barr 病毒（EBV）相关的 HLH 病例，包括其典型的诊断挑战和相关的高死亡率。某些诊断标准和在线工具可能有助于早期作出推测性诊断，从而加快治疗并带来更好的临床结果。
