Prognostic Factors Analysis of Children with Hemophagocytic Lymphohistiocytosis.
10.19746/j.cnki.issn.1009-2137.2021.06.046
- Author:
Qin LI
1
;
Yun-Sheng CHEN
2
;
Shi-Lin LIU
3
;
Si-Xi LIU
3
;
Xi-Min FANG
3
;
Wei-Guo YANG
4
;
Yue-Jie ZHENG
5
;
Wen-Jian WANG
5
;
Ji-Kui DENG
6
;
Jun YANG
7
;
Fei-Qiu WEN
3
;
Hui-Rong MAI
8
Author Information
1. Department of Hematological Oncology, Shenzhen Children's Hospital, China Medical University Shenzhen 518038, Guangdong Province, China.
2. Department of Medical Laboratory, Shenzhen Children's Hospital, Shenzhen 518038, Guangdong Province, China.
3. Department of Hematological Oncology, Shenzhen Children's Hospital, Shenzhen 518038, Guangdong Province, China.
4. Department of Critical Care Medicine Shenzhen Children's Hospital, Shenzhen 518038, Guangdong Province, China.
5. Department of Respiratory Medicine, Shenzhen Children's Hospital, Shenzhen 518038, Guangdong Province, China.
6. Department of Infectious Disease, Oncology, Shenzhen Children's Hospital, Shenzhen 518038, Guangdong Province, China.
7. Department of Rheumatology and Immunology Shenzhen Children's Hospital, Shenzhen 518038, Guangdong Province, China.
8. Department of Hematological Oncology, Shenzhen Children's Hospital, Shenzhen 518038, Guangdong Province, China,E-mail: maihuirong@163.com.
- Publication Type:Journal Article
- MeSH:
Adolescent;
Child;
Child, Preschool;
Humans;
Infant;
Lymphohistiocytosis, Hemophagocytic;
Prognosis;
Retrospective Studies;
Risk Factors;
Survival Rate
- From:
Journal of Experimental Hematology
2021;29(6):1957-1962
- CountryChina
- Language:Chinese
-
Abstract:
OBJECTIVE:To analyze the risk factors affecting prognosis of children with hemophagocytic lymphohistiocytosis (HLH).
METHODS:The clinical manifestations and laboratory data of 143 HLH children who met the HLH-2004 diagnostic criteria in Shenzhen Children's Hospital from January 2009 to May 2017 were retrospectively analyzed, and the independent factors affecting prognosis were also analyzed.
RESULTS:The median age of 143 HLH children was 1.9 (0.1-14.3) years old, and the median follow-up time was 6.7 years (1 day - 11.9 years). The overall survival rate of 1 month, 1 year, and 10 years was (87.4±5.5)%, (81.1±6.5)%, and (81.1±6.5)%, respectively. The deaths occurred within 1 year after onset. Multivariate analysis showed that central nervous system (CNS) involvement (P=0.047), low hemoglobin (P=0.002), prolonged activated partial thromboplastin time (APTT) (P<0.001), high triglyceride (P=0.005) were all the independent risk factors affecting survival of the children. Receiver operating characteristic curve indicated that APTT (AUC=0.753, P<0.001) was more valuable than other risk factors in predicting death of the children. The cut-off value of APTT was 56.6 s, and the sensitivity and specificity of which was 55.6% and 89.7%, respectively.
CONCLUSION:Hypohemoglobinemia, prolonged APTT, hypertriglyceridemia, and CNS involvement the risk factors affecting prognosis of HLH, and prolonged APTT shows a strong predictive value for death.
