Clinical study on factor Ⅷ inhibitor in children with hemophilia A.
10.3760/cma.j.issn.0253-2727.2020.02.010
- Author:
Bao Jun SHANG
1
,
2
;
Shi Wei YANG
1
,
3
,
4
;
Ping Chong LEI
5
,
6
;
Rong Jun MA
7
;
Xiang Dong HE
5
,
6
;
Xiao Li YUAN
7
;
Li JIANG
7
;
Yu Long LI
1
,
2
;
Xiao Yan DONG
1
,
2
;
Zhen WANG
7
;
Lin ZHANG
1
,
2
;
Zun Min ZHU
1
,
2
Author Information
1. Institute of Hematology of Henan Provincial People's Hospital, 450003, China
2. Henan Key laboratory of Stem Cell Differentiation and Modification, Zhengzhou 450003, China.
3. Henan Key laboratory of Stem Cell Differentiation and Modification, Zhengzhou 450003, China
4. Department of Hematology, Henan Provincial People' s Hospital/People's Hospital of Zhengzhou University, Zhengzhou 450003, China.
5. Department of Hematology, Henan Provincial People' s Hospital/People's Hospital of Zhengzhou University, Zhengzhou 450003, China
6. Henan Provincial Registration Management Center of Hemophilia, Zhengzhou 450003, China.
7. Department of Hematology, Henan Provincial People' s Hospital/People's Hospital of Zhengzhou University, Zhengzhou 450003, China.
- Publication Type:Journal Article
- Keywords:
Hemophilia A;
Hemophilic arthropathy;
Inhibitor;
Ultrasound score
- MeSH:
Child;
Factor VIII/therapeutic use*;
Hemarthrosis;
Hemophilia A/drug therapy*;
Hemorrhage;
Humans;
Retrospective Studies
- From:
Chinese Journal of Hematology
2020;41(2):138-142
- CountryChina
- Language:Chinese
-
Abstract:
Objective: To reveal the related factors of inhibitors and differences ofhemorrhage and joint disease before and after the production of inhibitors in children with hemophilia A (HA) . Methods: Retrospective analyses of the clinical data of 381 children with HA under the age of 16 registered in the Registration Management Center of Hemophilia in Henan Provincial from January 2015 to August 2018. Results: A total of the 381 children were enrolled with 116 (30.4%) mild, 196 (51.4%) moderate, and 69 (18.1%) severe cases; 54 patients (14.2%) had inhibitors, including 22 high and 32 low titer inhibitors. Positive family history was positively associated with inhibitors[P<0.001, OR=3.299 (95%CI 1.743-5.983) ], and high-intensity exposure was associated with inhibitors[P=0.002, OR=2.587 (95%CI 1.414-4.731) ]. High-intensity exposure was associated with high titer inhibitor production[P=0.001, OR=8.689 (95%CI 2.464-30.638) ], and high-intensity exposure increased the risk of high titer inhibitors in HA patients. After inhibitors occurred in 54 patients with HA, the rates of overall joint annual bleeding (z=-3.440, P=0.001) and traumatic annual bleeding (z=-2.232, P=0.026) increased, but the rates of the annual joint bleeding (z=-1.342, P=0.180) and spontaneous annual bleeding (z=-1.414, P=0.157) remained to be not statistically significant. The joint ultrasound score did not change significantly after the inhibitor information (z=-0.632, P=0.527) . Conclusions: Positive family history and high-intensity exposure could increase the risk of F Ⅷ inhibitors in HA patients, and high-intensity exposure increased the risk of high titer inhibitors. The rates of the overall joint annual bleeding and traumatic annual bleeding increased after the inhibitor information.
