噬血细胞淋巴组织细胞增生症是一种威胁生命的疾病,其特征是细胞毒性T淋巴细胞的持续病理激活,自然杀伤细胞,和巨噬细胞。我们介绍了一名高烧的年轻患者的详细信息,黄疸,和呼吸困难。关于调查,他得了肝炎,贫血,中性粒细胞减少症,和凝血病。他也有高甘油三酯血症,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生症,经颈静脉肝活检显示坏死性肉芽肿在抗酸杆菌染色上为结核分枝杆菌阳性。他通过免疫抑制剂和抗结核治疗的组合成功治疗。结核相关性噬血细胞综合征是罕见的,应考虑在患者的原因不明的噬血细胞综合征,尤其是在结核病流行地区。及时识别和抗结核治疗和免疫抑制剂治疗与良好的预后相关。
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 Mycobacterium tuberculosis 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.