Smith Kinaya K, Hobbs Linzi M, Narra Ravi
Department of Internal Medicine, Medical College of Wisconsin, Milwaukee, USA.
Department of Hematology and Oncology, Medical College of Wisconsin, Milwaukee, USA.
Cureus. 2024 Nov 2;16(11):e72884. doi: 10.7759/cureus.72884. eCollection 2024 Nov.
Primary hemophagocytic lymphohistiocytosis (HLH) is a rare, often fatal immune disorder characterized by an overactivation of the immune system. This disease is more common in children but has been known to occur in the occasional adult. The criteria for diagnosis in children do not correlate well with diagnosis in adults, and the numerous variations of presentation in adults often lead to a delay in diagnosis and treatment initiation. This case report highlights an example of primary HLH in a 41-year-old female patient who experienced a delay in diagnosis and appropriate treatment due to the similarity of her early symptoms to more common conditions. Delays in diagnosis and treatment can lead to poor outcomes, including death. Thus, it is important for providers to be able to recognize the varying signs and symptoms of primary HLH in an adult to ensure treatment is initiated as early as possible in the disease course.
原发性噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见的、通常致命的免疫紊乱疾病，其特征为免疫系统过度激活。这种疾病在儿童中更为常见，但也有成年人偶发的情况。儿童的诊断标准与成人的诊断标准相关性不佳，而且成人中众多的表现形式常常导致诊断和治疗开始的延迟。本病例报告突出了一名41岁女性患者原发性HLH的例子，该患者由于其早期症状与更常见病症相似而经历了诊断和适当治疗的延迟。诊断和治疗的延迟可能导致不良后果，包括死亡。因此，医疗人员能够识别成人原发性HLH的各种体征和症状，以确保在疾病进程中尽早开始治疗，这一点很重要。
