Department of Hematology, Odense University Hospital, Odense, Syddanmark, Denmark.
Department of Clinical Research, University of Southern Denmark, Odense, Syddanmark, Denmark.
BMJ Case Rep. 2024 Sep 25;17(9):e261936. doi: 10.1136/bcr-2024-261936.
Haemophagocytic lymphohistiocytosis (HLH) is a syndrome with an abnormal activation of the immune system and is associated with a high mortality even with treatment. We present a case of a woman in her mid-50s who developed HLH triggered by miliary tuberculosis (TB) while receiving a tumour necrosis factor alpha inhibitor.The patient was admitted with a high fever and respiratory pain. Her condition deteriorated despite empirical treatment. Diagnosis of HLH was established based on clinical presentation, H-score and HLH-04 criteria. Concurrently, miliary TB was identified as the trigger. She was treated with anti-tuberculous therapy and HLH-directed treatment with dexamethasone, etoposide and anakinra. Initial improvement was observed, leading to the withholding of HLH-orientated treatment. However, several relapses occurred, necessitating prolonged HLH treatment.A literature review corroborated the importance of combined anti-tuberculous and immunosuppressive therapy for managing HLH. This case underscores the necessity of timely and comprehensive management of HLH-oriented treatment.
噬血细胞性淋巴组织细胞增生症（HLH）是一种免疫系统异常激活的综合征，即使经过治疗，死亡率仍然很高。我们报告了一例 50 多岁的女性患者，在接受肿瘤坏死因子-α抑制剂治疗的同时，因粟粒性肺结核（TB）而引发 HLH。该患者因高热和呼吸疼痛入院。尽管进行了经验性治疗，但病情仍在恶化。根据临床表现、H 评分和 HLH-04 标准，诊断为 HLH。同时，粟粒性 TB 被确定为诱因。她接受了抗结核治疗和 HLH 靶向治疗，包括地塞米松、依托泊苷和阿那白滞素。观察到初始改善，因此停止了 HLH 靶向治疗。然而，出现了多次复发，需要延长 HLH 治疗。文献复习证实了联合抗结核和免疫抑制治疗对 HLH 管理的重要性。该病例强调了及时和全面管理 HLH 靶向治疗的必要性。
