Advances in adult hemophagocytic lymphohistiocytosis
10.3760/cma.j.issn.1009-9921.2016.07.017
- VernacularTitle:成年人嗜血细胞性淋巴组织细胞增多症研究进展
- Author:
Ting XU
;
Depei WU
- Publication Type:Journal Article
- Keywords:
Lymphohistiocytosis,hemophagocytic;
Adult;
Diagnosis;
Therapy
- From:
Journal of Leukemia & Lymphoma
2016;25(7):434-438
- CountryChina
- Language:Chinese
-
Abstract:
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by extreme immune activation, resulting in pathologic inflammation. The HLH-2004 diagnostic criteria is used widely based on the data from pediatric patients. However, there are differences between children and adults in the etiology, clinical and laboratory characteristics for HLH. Given these differences, diagnostic scoring systems unique to adult HLH have been proposed. HLH in adults is a much more heterogeneous syndrome requiring a more individualized approach depending on the underlying trigger, disease severity, as well as genetic background.