Pediatric macrophage activation syndrome/sepsis-associated hemophagocytic lymphohistiocytosis: a diagnostic and therapeutic challenge
10.3760/cma.j.issn.1673-4912.2013.03.002
- VernacularTitle:挑战:巨噬细胞活化综合征和脓毒症相关噬血细胞淋巴组织细胞增生症
- Author:
Jian HU
- Publication Type:Journal Article
- Keywords:
Hemophagocytic lymphohistiocytosis;
Macrophage activation syndrome;
Sepsis;
Still' s disease;
Systemic lupus erythematosus;
Children
- From:
Chinese Pediatric Emergency Medicine
2013;20(3):236-239
- CountryChina
- Language:Chinese
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Abstract:
It is an important turning point that early diagnosis and prompts initiation of adequate treatment improve the outcomes of primary or secondary hemophagocytic lymphohistiocytosis (HLH) which is a rare life-threatening syndrome.Even though the survival rate of the patients with HLH has been improved because of the immunochemotherapy or targeted immunotherapy based on new findings in the immunopathogenesis and the genetic defects,reducing the mortality will be main challenge for the pediatricians in pediatric intensive care unit.All of the clinical features are significant clues for the diagnosis,including continuing fever,specific liver dysfunction with coagulation abnormalities,pancytopenia,hypertriglyceridemia and hyperferritinemia.Defining patients' natural killer cells status during the different stages of sepsis and autoimmune diseases will be guiding innovative immunointerventions.
