Advances in etiology and pathogenesis of hemophagocytic syndrome
- VernacularTitle:噬血细胞综合征病因及发病机制研究进展
- Author:
Fangqiu LI
;
- Publication Type:Journal Article
- Keywords:
Hemophagocytic syndrome (HPS);
Etiology;
Pathogenesis;
Cytotoxicity;
Perforin
- From:
Journal of Medical Postgraduates
2003;0(10):-
- CountryChina
- Language:Chinese
-
Abstract:
Hemophagocytic syndrome (HPS) is a clinicopathologic entity occurs as a consequence of uncontrolled dysregulated cellular immune reactivity caused by a number of different underlying diseases,characterized by hypercytokinemia and systemic proliferation of benign hemophagocytic cells. Hemophagocytic syndrome embraces both primary/inherited HPS and secondary/reactive HPS. Mutations in perforin gene have been identified in some inherited HPS patients,there may be other genetic abnormalities also responsible for the disease. The causes of secondary HPS are diverse,including infections,malignancy and other unknown underlying diseases. A great advance in pathogenesis of HPS has been made and the results of recent research suggest that onset of HPS is closely associated with deficiency in lymphocyte mediated cytotoxicity and lowing apoptosis of activated immune cells.
