Elhence Anshuman, Aggarwal Abhinav, Goel Ashish, Aggarwal Mukul, Das Prasenjit
Department of Gastroenetrology, All India Institute of Medical Sciences, New Delhi, India.
Department of Medicine, University College of Medical Sciences, India.
J Clin Exp Hepatol. 2021 Jan-Feb;11(1):149-153. doi: 10.1016/j.jceh.2020.05.007. Epub 2020 May 22.
Hemophagocytic lymphohistiocytosis is a life-threatening disorder characterized by persistent pathologic activation of cytotoxic T lymphocytes, natural killer cells, and macrophages. We present details of a young patient who presented with high-grade fever, jaundice, and breathlessness. On investigations, he had hepatitis, anemia, neutropenia, and coagulopathy. He also had hypertriglyceridemia, hypofibrinogenemia, and hyperferritinemia. Bone marrow aspiration revealed histiocytosis, and transjugular liver biopsy revealed necrotizing granulomas positive for on acid-fast bacilli staining. He was successfully managed with a combination of immunosuppressants and antitubercular therapy. Tuberculosis associated hemophagocytosis syndrome is rare and should be considered in patients with unexplained hemophagocytosis syndrome, especially in tuberculosis-endemic regions. Prompt recognition and treatment with antitubercular treatment and immunosuppressants are associated with good outcomes.
噬血细胞性淋巴组织细胞增生症是一种危及生命的疾病，其特征为细胞毒性T淋巴细胞、自然杀伤细胞和巨噬细胞持续发生病理性激活。我们报告一名年轻患者的详细情况，该患者出现高热、黄疸和呼吸急促。经检查，他患有肝炎、贫血、中性粒细胞减少症和凝血病。他还患有高甘油三酯血症、低纤维蛋白原血症和高铁蛋白血症。骨髓穿刺显示组织细胞增多症，经颈静脉肝活检显示坏死性肉芽肿，抗酸杆菌染色呈阳性。他通过免疫抑制剂和抗结核治疗联合成功治愈。结核相关噬血细胞综合征罕见，对于不明原因的噬血细胞综合征患者应予以考虑，尤其是在结核病流行地区。及时识别并用抗结核治疗和免疫抑制剂进行治疗可带来良好预后。
