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噬血细胞性淋巴组织细胞增生症是血液科的危重症综合征,儿童患者较多。本症为一种综合征,有多种致病因素,包括先天性疾病和后天性疾病。在儿童患者中病毒感染,尤其是EB病毒感染是本病常见病因。其他病因如肿瘤相关、结缔组织病相关(巨噬细胞活化综合征)也不能忽视,其诊断标准也有所不同。2004年国际组织细胞协会规范了本综合征的诊断标准。在临床中识别病因、进行诊断十分重要。
Hemophagocytic lymphohistiocytosis is a critically ill syndrome of hematology, with more children. The disease is a syndrome, a variety of risk factors, including congenital and acquired diseases. In children with viral infections, especially EB virus infection is a common cause of this disease. Other causes such as tumor-related, connective tissue disease (macrophage activation syndrome) can not be ignored, the diagnostic criteria are also different. 2004 International Association of Cell Standards regulate the diagnostic criteria for this syndrome. In the clinical identification of the cause, the diagnosis is very important.