原发性噬血细胞性淋巴组织细胞增生症的病因和发病机制研究进展
被引量:6
Advances in the Etiology and Pathogenesis of Primary Hemophagocytic Lymphohistiocytosis
摘要
原发性噬血细胞性淋巴组织细胞增生症(Primary hemophagocytic lymphohistiocytosis,PHLH)为常染色体或x连锁隐性遗传病,是一组由各种致病因素导致机体免疫功能紊乱引起单核/巨噬细胞系统反应性增生、释放大量细胞因子、引起多脏器浸润及全血细胞减少为特征的疾病。本病主要表现为高热、肝脾肿大、全血细胞减少、噬血细胞增多、高甘油三酯血症、
出处
《儿科药学杂志》
CAS
2012年第6期50-56,共7页
Journal of Pediatric Pharmacy
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