Khreisat Ali, Maria Inna Mikaella Sta, Grasso-Knight Giovi, Mansour Meghan
Corewell Health William Beaumont University Hospital, Royal Oak, MI.
Oakland University William Beaumont School of Medicine, Rochester Hills, MI.
HCA Healthc J Med. 2024 Dec 1;5(6):745-749. doi: 10.36518/2689-0216.1741. eCollection 2024.
Hemophagocytic lymphohistiocytosis (HLH) is a non-neoplastic proliferation and macrophage activation that induces cytokine-mediated bone marrow suppression and features of intense phagocytosis in the bone marrow and liver, leading to multi-organ dysfunction and ultimate failure. The diagnosis of HLH in an intensive care setting is challenging, and it is associated with high morbidity and mortality. HLH-94 is the standard protocol for treatment, consisting of dexamethasone and chemotherapy like etoposide.
We present the case of a 73-year-old woman who had a prolonged hospitalization for vomiting, diarrhea, and dehydration. Her conditions were complicated by acute refractory pancytopenia on the 12th day of admission, leading to multi-organ failure, including anuric renal failure requiring renal replacement therapy and respiratory failure requiring intubation. After a thorough workup, she was diagnosed with HLH using HLH-2004 diagnostic criteria and confirmed by a bone marrow biopsy. She was started on supportive therapy and high-dose intravenous dexamethasone with an appropriate clinical response. Her pancytopenia improved, and she no longer required ventilator support for respiratory failure or dialysis for renal failure. Unfortunately, her hospital course was complicated by a sentinel event leading to her death.
This case emphasizes that early recognition and treatment initiation of HLH are crucial to prevent adverse outcomes and mortality. Treatment should be tailored based on the underlying HLH trigger, as chemotherapy-based treatment regimens may result in overtreatment and unnecessary toxicities. Further studies are needed to increase clinicians' awareness and management of secondary cases of HLH.
噬血细胞性淋巴组织细胞增生症（HLH）是一种非肿瘤性增殖和巨噬细胞活化，可诱导细胞因子介导的骨髓抑制以及骨髓和肝脏中强烈吞噬作用的特征，导致多器官功能障碍和最终衰竭。在重症监护环境中诊断HLH具有挑战性，且其发病率和死亡率较高。HLH-94是标准治疗方案，由地塞米松和依托泊苷等化疗药物组成。
我们报告一例73岁女性患者，因呕吐、腹泻和脱水住院时间延长。入院第12天，她的病情并发急性难治性全血细胞减少症，导致多器官衰竭，包括需要肾脏替代治疗的无尿性肾衰竭和需要插管的呼吸衰竭。经过全面检查，根据HLH-2004诊断标准诊断她为HLH，并通过骨髓活检得以证实。她开始接受支持治疗和大剂量静脉用地塞米松治疗，临床反应良好。她的全血细胞减少症有所改善，不再需要呼吸机支持呼吸衰竭或透析治疗肾衰竭。不幸的是，她的住院过程因一次严重事件而复杂化，最终导致死亡。
本病例强调HLH的早期识别和治疗启动对于预防不良后果和死亡至关重要。应根据潜在的HLH触发因素调整治疗方案，因为基于化疗的治疗方案可能导致过度治疗和不必要的毒性反应。需要进一步研究以提高临床医生对HLH继发性病例的认识和管理水平。
