Pinheiro Ramos Rafael, Névoa Joana, Campos Cunha João, Sarmento Gonçalo
Department of Internal Medicine, Unidade Local de Saúde Entre Douro e Vouga, Santa Maria da Feira, PRT.
Cureus. 2024 Dec 7;16(12):e75279. doi: 10.7759/cureus.75279. eCollection 2024 Dec.
Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal entity characterized by an unregulated activation of the immune system. In the adult population, it is most commonly secondary to infectious, autoimmune, or neoplastic diseases. We present a case of a 23-year-old female diagnosed with infectious mononucleosis and hospitalized due to a persistent three-week fever and malaise with a new onset of jaundice and findings compatible with acute hepatitis and hepatosplenomegaly. Due to a lack of response to standard supportive care, maintained fever, persistent analytical worsening (hyperferritinemia, bicytopenia, cytocholestasis, and hypertriglyceridemia), and hemophagocytocic phenomena on myelogram, the diagnosis of HLH secondary to Epstein-Barr virus infection was attained. Treatment with systemic corticosteroids was initiated with a continuously favorable evolution. This case aimed to describe and highlight the complexity of the diagnostic approach and the urgency of directed therapeutic attitudes in the management of HLH.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见但可能致命的疾病，其特征为免疫系统的失控激活。在成人中，它最常见于继发于感染性、自身免疫性或肿瘤性疾病。我们报告一例23岁女性病例，该患者被诊断为传染性单核细胞增多症，因持续三周发热、乏力，并伴有新发黄疸以及符合急性肝炎和肝脾肿大的表现而住院。由于对标准支持治疗无反应、持续发热、持续的分析指标恶化（高铁蛋白血症、双血细胞减少、细胞性胆汁淤积和高甘油三酯血症）以及骨髓检查出现噬血细胞现象，最终诊断为继发于爱泼斯坦 - 巴尔病毒感染的HLH。开始使用全身性皮质类固醇治疗，病情持续好转。本病例旨在描述和强调HLH诊断方法的复杂性以及在其管理中采取针对性治疗态度的紧迫性。
