Comparative analysis of childhood macrophage activation syndrome and other hemophagocytic syndrome
10.3760/cma.j.issn.1007-7480.2010.07.010
- VernacularTitle:儿童巨噬细胞活化综合征与其他噬血细胞综合征的临床比较分析
- Author:
Yucan ZHENG
;
Xiaoqing QIAN
;
Juan LI
;
Yayuan ZHANG
;
Haiguo YU
- Publication Type:Journal Article
- Keywords:
Histiocytosis,non-Langerhans-cell;
Child;
Macrophage activation syndrome
- From:
Chinese Journal of Rheumatology
2010;14(7):477-479
- CountryChina
- Language:Chinese
-
Abstract:
Objective To compare the clinical features, diagnosis, treatment and prognosis between macrophages activation syndrome (MAS) and other hemophagocytic syndrome (HPS). Methods Thirty-six children with HPS were identified at Nanjing Children's Hospital during January 2006 to March 2009. They could be classi-fied into MAS group (13 patients) and other HPS group (23 patients). All relevant clinical features, laboratory data, treatments and outcomes were analyzed with t test,χ2 test and Fisher's exact test.Results Patients with MAS tended to be elder than those with other HPSs [(7.7±1.3) years vs (2.6±0.5)years, t=3.899, P=0.004]. There was no difference in gender distribution. In MAS cases, the central nervous system (69% vs 13%, P=0.001), circulatory system (23% vs 9%, P=0.047) and the urinary system (38% vs 9%, P=0.033) were usually involved. The clinical symptoms of MAS were more sever than other HPS. Serum ferritin [(9703±9819) μg/L vs (4569±1396) μg/L, t=2.854, P=0.015] and erythrocyte sedimentation rate (ESR) [(53±32) mm/1 h vs (20±14) mm/1 h, t=2.708, P=0.020] changed more obviously in MAS cases compared with other HPS. Conclusion, Childhood MAS is different from other HPS in terms of age, etiology, clinical manifestations, laboratory tests and treatments.
