Department of Otorhinolaryngology-Head and Neck Surgery, Kyungpook National University School of Medicine, Daegu, Korea.
Department of Pediatrics, School of Medicine, Kyungpook National University, Daegu, Korea.
Medicine (Baltimore). 2024 Jun 21;103(25):e38616. doi: 10.1097/MD.0000000000038616.
Hemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening syndrome for which early recognition and treatment are essential for improving outcomes. HLH is characterized by uncontrolled immune activation leading to fever, cytopenias, hepatosplenomegaly, coagulation abnormalities, and elevated typical markers. This condition can be genetic or secondary, with the latter often triggered by infections. Here, we present a unique case of HLH secondary to acute otitis media (AOM), a common ear infection.
We describe a 4-year-old boy who initially presented with a high fever and otalgia, later diagnosed with bilateral AOM. Despite antibiotic treatment, his condition deteriorated.
The patient fulfilled diagnostic criteria for HLH.
Aggressive treatment by using combination therapy with immunoglobulins, intravenous steroids (dexamethasone), cyclosporine, and etoposide was performed.
After 1 month of treatment, improvement in the otologic symptoms was observed, and hematological findings gradually improved and normalized.
The link between AOM and HLH may be associated with inflammatory responses and immunological mechanisms, highlighting the importance of considering HLH in severe infection cases. This case emphasizes the need for prompt diagnosis and management, especially in secondary HLH scenarios, to improve patient outcomes. It is imperative to be aware of the potential correlation between these 2 conditions, and healthcare professionals should consider the likelihood of HLH.
噬血细胞性淋巴组织细胞增生症(HLH)是一种潜在危及生命的综合征,早期识别和治疗对于改善预后至关重要。HLH 的特征是免疫激活失控,导致发热、血细胞减少、肝脾肿大、凝血异常和典型标志物升高。这种情况可以是遗传性的,也可以是继发性的,后者通常由感染引发。在这里,我们报告了一例由急性中耳炎(AOM)引起的 HLH 病例,AOM 是一种常见的耳部感染。
我们描述了一位 4 岁男孩,最初表现为高热和耳痛,后来被诊断为双侧 AOM。尽管接受了抗生素治疗,但病情仍恶化。
该患者符合 HLH 的诊断标准。
采用免疫球蛋白、静脉注射类固醇(地塞米松)、环孢素和依托泊苷联合治疗进行积极治疗。
经过 1 个月的治疗,耳部症状有所改善,血液学发现逐渐改善并恢复正常。
AOM 和 HLH 之间的联系可能与炎症反应和免疫机制有关,这突出了在严重感染病例中考虑 HLH 的重要性。该病例强调了及时诊断和管理的必要性,尤其是在继发性 HLH 情况下,以改善患者的预后。必须意识到这两种情况之间存在潜在的相关性,医护人员应考虑 HLH 的可能性。