摘要
噬血细胞综合征（hemophagocytic lymphohistiocytosis，HLH）是一组以良性巨噬细胞增生和活化，伴随吞噬血细胞现象为特征的综合征。发病机制为自然杀伤细胞和细胞毒性T细胞介导的穿孔素依赖的细胞毒作用缺焰所导致的多系统炎症反应，临床主要特征为持续高热、血细胞减少、肝脾肿大、肝炎。原发性HLH治疗主要为抑制高炎症反应，包括类固醇激素和依托泊苷，以及胸腺肽的积极应用，必要时可行造血干细胞移植，继发性HLH应控制原发感染。
Hemophagocytic lymphohistiocytosis （HLH） is characterized by hemophagocytosis and a reactive process resulting from prolonged and excessive activation of antigen presenting ceils （macrophages, histi- ocytes） and CD8 ＋ T cells. The majority of genetic causes identified to date affect the cytotoxic function of NK and T cells, crippling immunologic mechanisms that mediate natural immune contraction. The predominant clini- cal findings of HLH are fevers （ often hectic and persistent）, cytopenias, hepatitis and splenomegaly. Due to the life-threatening implications of the diagnosis of genetically determined HLH, antiinflammatory therapy, often consisting of steroids, etoposide or antithymocyte globulin, should be instituted promptly, followed by curative hematopoietic cell transplantation. Secondary HLH, associated with autoimmune disorders or viral infections in teens and adults, should control the primary inflammatory.
出处
《国际儿科学杂志》
2012年第3期264-266,270,共4页
International Journal of Pediatrics
关键词
噬血细胞综合征
自然杀伤细胞
细胞毒性T细胞
Hemophagocytic lymphohistiocytosis
Natural killer cell
Cytotoxic lymphocyte
