School of Medicine, The George Washington University School of Medicine and Health Sciences, Washington, District of Columbia, USA
Division of Infectious Diseases, Children's National Hospital, Washington, District of Columbia, USA.
BMJ Case Rep. 2024 Jun 6;17(6):e260060. doi: 10.1136/bcr-2024-260060.
Haemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening hyperinflammatory syndrome characterised by persistent fevers, cytopenia, hepatosplenomegaly and systemic inflammation. Secondary HLH can be triggered by various aetiologies including infections, malignancies and autoimmune conditions. We highlight the complexity of HLH diagnosis and management by describing a case of an adolescent Salvadoran immigrant with HLH, newly diagnosed HIV, and disseminated histoplasmosis. The patient presented with neurological and ocular findings along with persistent fevers and cytopenia. He was diagnosed with HLH and treated with anakinra in addition to receiving treatment for HIV, and histoplasmosis. The patient's HLH resolved without corticosteroids or chemotherapy, which are considered the mainstays for HLH treatment. This case underscores the need for the evaluation and management of multiple infections and individualised management in patients presenting with HLH to achieve favourable outcomes.
噬血细胞性淋巴组织细胞增生症(HLH)是一种罕见的、危及生命的过度炎症综合征,其特征为持续发热、血细胞减少、肝脾肿大和全身炎症。继发性 HLH 可由多种病因引发,包括感染、恶性肿瘤和自身免疫性疾病。我们通过描述一名患有 HLH、新诊断 HIV 感染和播散性组织胞浆菌病的萨尔瓦多裔青少年患者的病例,强调了 HLH 诊断和管理的复杂性。该患者出现了神经和眼部表现,伴有持续发热和血细胞减少。他被诊断为 HLH,并接受了 anakinra 治疗,同时还接受了 HIV 和组织胞浆菌病的治疗。该患者的 HLH 在未使用皮质类固醇或化疗的情况下得到缓解,而皮质类固醇或化疗被认为是 HLH 治疗的主要方法。该病例强调了在出现 HLH 的患者中需要评估和管理多种感染,并进行个体化管理,以实现良好的结局。