Clinical features and prognosis of 28 children with hemophagocytic syndrome
10.3969/j.issn.1000-3606.2014.05.007
- VernacularTitle:儿童噬血细胞综合征28例临床特点及预后分析
- Author:
Dongqing XU
;
Xiaojun YUAN
;
Xia AN
;
Mengjie TANG
;
Chen WANG
- Publication Type:Journal Article
- Keywords:
hemophagocytic syndrome;
clinical features;
prognosis;
children
- From:
Journal of Clinical Pediatrics
2014;(5):425-429
- CountryChina
- Language:Chinese
-
Abstract:
Objectives To investigate the clinical profile and prognosis of hemophagocytic syndrome (HPS). Methods A retrospective study was carried out to analyze the clinical features and laboratory findings in 28 children with HPS. Fisher's exact probability method and Logistic multivariate regression were used to explore the prognostic risk factors.. Results HPS was clinically characterized by prolonged fever (100%), hepatomegaly (64.29%),and other minor features including respiratory symptoms (53.57%), splenomegaly (50%), hydrops of multiple serous cavity (42.86%), lymphadenectasis (32.14%), jaundice (17.85%), skin rash (14.29%), central nervous system involvement (14.29%), and alimentary tract hemorrhage (10.71%). Labo-ratory data showed that 1iver dysfunction, pancytopenia, coagulation abnormalities, disseminated intravascular coagulation, hy-pertriglyceridemia, decreased number of natural killer cells and hyponatremia were prominent. The etiological analysis indicated that infection associated hemophagocytic syndrome was most common (60.71%), in which EB virus associated HPS was pre-dominant, accounting for 64.71%. Significant difference was observed in the difference of albumin,blood urea nitrogen and acti-vated partial thromboplastin time between death and survival cases (P<0.05). The Logistic regression multivariate analysis showed that hypoalbuminemia was an independent prognostic factor. Conclusions There are various underlying diseases and clinical manifestations for HPS. The lower level of serum albumin is an independent prognostic factor. A prompt diagnosis and treatment is very important for HPS prognosis due to the rapid progression and high mortality.
