摘要
噬血细胞综合征以免疫调节功能异常导致的细胞因子风暴和免疫介导的多器官系统损伤为特征,临床上主要表现为发热,肝、脾肿大,全血细胞减少,高甘油三酯血症和/或低纤维蛋白原血症,肝、脾、骨髓等病理检查可发现噬血现象。该病起病急,且死亡率高,一般常继发于各种免疫功能低下状态、恶性肿瘤和感染患者。本文报道了1例肝脏结核继发噬血细胞综合征患者,通过骨髓穿刺及肝脏组织学活检确诊为噬血细胞综合征、肝结核。希望通过该病例提高临床医师对本病的认识。
Hemophagocytic syndrome(HPS)is characterized by cytokine storm and immune-mediated multi-organ system damage caused by abnormal immune regulation function.The main clinical manifestations are fever,hepatomegaly,splenomegaly,pancytopenia,hypertriglyceridemia and/or hypofibrinogenemia,hemophagocytosis evident on pathological examination(liver,spleen,bone marrow).The disease has an acute onset and high mortality rate,and is often secondary to patients with various immunocompromised states,malignant tumors,and infections.We described a case of HPS associated with hepatic tuberculosis.The patient was diagnosed with HPS and hepatic tuberculosis through bone marrow puncture and liver histological biopsy.It is hoped that this case will improve clinicians′understanding of this disease.
作者
胡想金
陈衍
周志慧
HU Xiangjin;CHEN Yan;ZHOU Zhihui(Department of Hepatology and Infectious Diseases,Zhejiang University School of Medicine Sir Run Run Shaw Hospital,Hangzhou 310016,China)
出处
《胃肠病学和肝病学杂志》
2026年第2期310-312,共3页
Chinese Journal of Gastroenterology and Hepatology
关键词
肝脏结核
噬血细胞综合征
死亡
Hepatic tuberculosis
Hemophagocytic syndrome
Death
