Agyeman Walter Y, Waitimu Susan, Seffah Kofi D, Allihien Saint-Martin, Soleye Saheed
Internal Medicine, Piedmont Athens Regional Medical Center, Athens, USA.
Cureus. 2023 Aug 21;15(8):e43875. doi: 10.7759/cureus.43875. eCollection 2023 Aug.
Hemophagocytic lymphohistiocytosis (HLH) is a rare and often missed presentation in the hospital setting. It is a hyperinflammatory syndrome of immune activation and dysregulation characterized by fever, cytopenias, elevated serum ferritin levels, and hepatosplenomegaly. It has a multifactorial etiology occurring primarily secondary to infection, malignancy, immune checkpoint inhibitors, or autoimmune disease. HLH that occurs in autoimmune diseases such as adult-onset Still's disease and systemic lupus erythematosus is referred to as macrophage activation syndrome. However, it may rarely be a primary disorder from a genetic defect. The pathophysiology of HLH is poorly understood and often results in multiorgan dysfunction. We present an older adult male patient with several hospital stays due to his symptoms, who presented with lethargy, low-grade fever, cough, dyspnea, and recurrent pleural effusions. He had bicytopenia, elevated ferritin, and hypertriglyceridemia. The diagnosis of HLH may be delayed, especially in older adult patients with an insidious course, and requires a high index of suspicion.
噬血细胞性淋巴组织细胞增生症（HLH）在医院环境中是一种罕见且常被漏诊的病症。它是一种免疫激活和失调的高度炎症综合征，其特征为发热、血细胞减少、血清铁蛋白水平升高以及肝脾肿大。它有多种病因，主要继发于感染、恶性肿瘤、免疫检查点抑制剂或自身免疫性疾病。在自身免疫性疾病如成人斯蒂尔病和系统性红斑狼疮中发生的HLH被称为巨噬细胞活化综合征。然而，它很少可能是由基因缺陷引起的原发性疾病。HLH的病理生理学知之甚少，常导致多器官功能障碍。我们报告一名老年男性患者，因症状多次住院，表现为嗜睡、低热、咳嗽、呼吸困难和反复胸腔积液。他有双血细胞减少、铁蛋白升高和高甘油三酯血症。HLH的诊断可能会延迟，尤其是在病程隐匿的老年患者中，需要高度怀疑。
