Abou-El-Hassan Hadi, Kantono Melvin, Bhagat Ankur, Hu Janie, Karp David, Jerome Renard, Randhawa Johnny S, Shafer Drake, Farmand Farbod
Neurology, Arrowhead Regional Medical Center, Colton, USA.
Internal Medicine, Arrowhead Regional Medical Center, Colton, USA.
Cureus. 2023 Mar 15;15(3):e36199. doi: 10.7759/cureus.36199. eCollection 2023 Mar.
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome characterized by a pathologic immune response in the setting of infection, malignancy, acute illness, or any immunological stimulus. Infection is the most common etiology of HLH. HLH involves aberrant activation of lymphocytes and macrophages with resultant hypercytokinemia due to an inappropriately stimulated and ineffective immune response. Here, we present the case of a previously healthy 19-year-old male presenting with hiccups and scleral icterus, who was found to have HLH due to a severe Epstein-Barr virus infection. Despite a morphologically normal bone marrow biopsy, the patient met the diagnostic criteria for HLH, including a low natural killer cell count and elevated soluble interleukin-2 receptor. Notably, ferritin was severely elevated at 85,810 ng/mL. The patient was treated with an induction course of dexamethasone intravenously for eight weeks. Since HLH can progress into multi-organ failure, timely diagnosis and prompt initiation of treatment are critical. Novel disease-modifying therapies and further clinical trials are warranted to treat this potentially fatal immunological disease with multisystem ramifications.
噬血细胞性淋巴组织细胞增生症(HLH)是一种危及生命的高炎症综合征,其特征是在感染、恶性肿瘤、急性疾病或任何免疫刺激情况下出现病理性免疫反应。感染是HLH最常见的病因。HLH涉及淋巴细胞和巨噬细胞的异常激活,由于免疫反应刺激不当且无效,导致细胞因子血症。在此,我们报告一例既往健康的19岁男性病例,该患者出现打嗝和巩膜黄疸,经检查发现因严重的爱泼斯坦-巴尔病毒感染而患有HLH。尽管骨髓活检形态正常,但该患者符合HLH的诊断标准,包括自然杀伤细胞计数低和可溶性白细胞介素-2受体升高。值得注意的是,铁蛋白严重升高,达85810 ng/mL。该患者接受了为期八周的静脉注射地塞米松诱导治疗。由于HLH可进展为多器官功能衰竭,及时诊断和迅速开始治疗至关重要。需要新型疾病修正疗法和进一步的临床试验来治疗这种具有多系统影响的潜在致命性免疫疾病。