摘要
噬血细胞综合征是一类免疫系统功能紊乱所致的疾病,其病理生理机制是免疫缺陷和免疫调节异常,导致淋巴细胞和组织细胞过度活化,产生大量炎性因子,从而引起危及生命的过度炎性反应。临床表现为高热,肝脾、淋巴结肿大,全血细胞减少,肝功能异常和凝血功能障碍,按病因可分为遗传性和继发性两大类,其临床表现复杂,起病急骤,容易误诊。
Hemophagocytic syndrome is a kind of syndrome caused by immune system dysfunction. Its pathophysiology is immune deficiency and immune dysregulation, which leads to the excessive activation of lymphocytes and histiocytes and production of large amounts of inflanmmtory cytokines, causing life-threatening excessive inflammatory response. Its clinical manifestations are fever, enlargement of liver, spleen and lymph nodes, pancytopenia, abnormal liver function and coagulation disorders. According to etiology,hemophagocytic syndrome can be divided into hereditary kind and secondary kind. Due to its complex clinical manifestationsand raoid onset,it is easy to be misdiagnosed.
出处
《医学综述》
2014年第17期3192-3194,共3页
Medical Recapitulate
关键词
噬血细胞综合征
儿童
免疫
Hemophagocytic syndrome
Children
Immunization
