Brahmbhatt Niravkumar K, Pinson-Hilkemeyer Amber, Narang Saransh, Patel Tirth M, Bhojwani Deesha, Patil Sachin M, Roland William
Internal Medicine, University of Missouri Health Care, Columbia, USA.
Internal Medicine, University of Missouri Healthcare, Columbia, USA.
Cureus. 2024 Sep 12;16(9):e69294. doi: 10.7759/cureus.69294. eCollection 2024 Sep.
Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening disorder marked by excessive immune system activation. Diagnosing HLH is particularly difficult due to its diverse etiology and similar clinical presentation. Patients with HLH often require intensive critical care. The condition is divided into HLH syndrome (genetic defects) and HLH disease, and both can be triggered by factors such as infections, autoimmune diseases, or malignancies. We present here the first documented case of acute HLH secondary to tularemia with septic shock in a 62-year-old female. Initially suspected due to malignancy-induced acute HLH, she was treated with etoposide and dexamethasone and was immediately transitioned to antimicrobials once she was confirmed positive for on blood cultures at the state microbiology laboratory. She had a prolonged clinical course of 31 days, followed by an excellent recovery after treatment with antimicrobials. This case highlights the importance of recognizing tularemia as a potential cause of HLH. Identifying and addressing the causative agent promptly is crucial for effective management and treatment of HLH.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见的、危及生命的疾病，其特征是免疫系统过度激活。由于其病因多样且临床表现相似，HLH的诊断尤为困难。HLH患者通常需要重症监护。该病症分为HLH综合征（遗传缺陷）和HLH疾病，两者均可由感染、自身免疫性疾病或恶性肿瘤等因素引发。我们在此报告首例62岁女性因兔热病继发急性HLH并伴有感染性休克的病例。最初因怀疑是由恶性肿瘤引起的急性HLH，她接受了依托泊苷和地塞米松治疗，一旦在州立微生物实验室的血培养中确诊阳性，她立即改用抗菌药物治疗。她的临床病程长达31天，经抗菌药物治疗后恢复良好。该病例凸显了认识到兔热病是HLH潜在病因的重要性。及时识别并处理病原体对于HLH的有效管理和治疗至关重要。
