Chand Abhimanyu, Malbul Kiran, Thapa Ashish, Shrestha Merry, Bhandari Santosh Bikram, Adhikari Prabhat
Sushma Koirala Memorial Hospital, Shankharapur.
Bir Hospital, Kathmandu.
Ann Med Surg (Lond). 2024 Jul 1;86(8):4918-4920. doi: 10.1097/MS9.0000000000002334. eCollection 2024 Aug.
Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory disorder characterized by fever, cytopenia, splenomegaly, and hemophagocytosis. Without prompt treatment, HLH can rapidly progress to life-threatening multiorgan failure. The authors present a case of occult HLH with severe bicytopenia and organ dysfunction requiring intensive care.
A 20-year-old male presented with fever, cough, and constitutional symptoms. He developed hypoxia, elevated transaminases, and bicytopenia. Despite transfusions, platelet counts remained critically low. With high suspicion for HLH, head computed tomography and bone marrow biopsy was although panned but couldn't be performed due to resource less settings. And with suspicion for HLH treatment with high-dose dexamethasone was initiated as counts improved.
The patient required mechanical ventilation for pulmonary infiltrates. He exhibited seizure activity and epistaxis related to coagulopathy. On hospital day 9, he was successfully extubated as counts normalized. He was discharged from the intensive care unit once stable.
This case illustrates a delayed diagnosis of HLH masquerading as a fever of unknown origin. HLH should be urgently considered in patients with unexplained cytopenia, organ dysfunction, and systemic inflammation. Early treatment with immunotherapy can be lifesaving, whereas delays may precipitate irreversible end-organ damage.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见的高炎症性疾病，其特征为发热、血细胞减少、脾肿大和噬血细胞现象。若不及时治疗，HLH可迅速发展为危及生命的多器官功能衰竭。作者报告一例隐匿性HLH病例，该患者有严重双系血细胞减少和器官功能障碍，需要重症监护。
一名20岁男性出现发热、咳嗽和全身症状。他出现了低氧血症、转氨酶升高和双系血细胞减少。尽管进行了输血，但血小板计数仍极低。由于高度怀疑HLH，虽安排了头部计算机断层扫描和骨髓活检，但因资源有限而未能进行。随着血细胞计数改善，鉴于怀疑HLH，开始使用大剂量地塞米松进行治疗。
患者因肺部浸润需要机械通气。他出现了与凝血病相关的癫痫发作和鼻出血。在住院第9天，随着血细胞计数恢复正常，他成功脱机。病情稳定后，他从重症监护病房出院。
本病例说明了HLH被误诊为不明原因发热的情况。对于不明原因血细胞减少、器官功能障碍和全身炎症的患者，应紧急考虑HLH。早期免疫治疗可挽救生命，而延误治疗可能导致不可逆的终末器官损伤。
