The explanation and analysis of 2009-hemophagocytic lymphohistiocytosis
10.3760/cma.j.issn.1673-4408.2012.03.015
- VernacularTitle:2009年噬血细胞综合征诊疗规范详解
- Author:
Juan WANG
;
Guimei GUO
- Publication Type:Journal Article
- Keywords:
Hemophagocytic lymphohistiocytosis;
Natural killer cell;
Cytotoxic lymphocyte
- From:
International Journal of Pediatrics
2012;39(3):264-266,270
- CountryChina
- Language:Chinese
-
Abstract:
Hemophagocytic lymphohistiocytosis (HLH) is characterized by hemophagocytosis and a reactive process resulting from prolonged and excessive activation of antigen presenting cells (macrophages,histiocytes) 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 clinical 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.
