Analysis of the effects of low/intermediate dose of coagulation factor Ⅷ on 30 adult patients with severe hemophilia A in a single center.
10.3760/cma.j.issn.0253-2727.2023.01.007
- VernacularTitle:单中心成人重型血友病A低/中剂量凝血因子Ⅷ预防治疗30例疗效分析
- Author:
Yan Hui YUAN
1
;
Pei Pei XU
1
;
Yue Yi XU
1
;
Sha LIU
2
;
Xiao Yan SHAO
1
;
Wei Jing ZHANG
2
;
Li GONG
2
;
Min ZHOU
1
;
Bing CHEN
1
;
Rong Fu ZHOU
1
Author Information
1. Department of Hematology, the Affiliated Drum Tower Hospital of Nanjing University Medical School, Nanjing 210008, China.
2. Department of Ultrasound, the Affiliated Drum Tower Hospital of Nanjing University Medical School, Nanjing 210008, China.
- Publication Type:Journal Article
- Keywords:
Coagulation factor Ⅷ;
Hemophilia A;
Prophylaxis
- MeSH:
Humans;
Hemophilia A/drug therapy*;
Factor VIII/therapeutic use*;
Quality of Life;
Retrospective Studies;
Hemarthrosis/prevention & control*;
Hemorrhage/drug therapy*
- From:
Chinese Journal of Hematology
2023;44(1):38-42
- CountryChina
- Language:Chinese
-
Abstract:
Objective: To evaluate the clinical effects of low- and intermediate-dose factor Ⅷ (F Ⅷ) prophylaxis in Chinese adult patients with severe hemophilia A. Methods: Thirty adult patients with severe hemophilia A who received low- (n=20) /intermediate-dose (n=10) F Ⅷ prophylaxis at Nanjing Drum Tower Hospital affiliated with Nanjing University Medical College were included in the study. The annual bleeding rate (ABR), annual joint bleeding rate (AJBR), number of target joints, functional independence score of hemophilia (FISH), quality of life score, and health status score (SF-36) before and after preventive treatment were retrospectively analyzed and compared. Results: The median follow-up was 48 months. Compared with on-demand treatment, low- and intermediate-dose prophylaxis significantly reduced ABR, AJBR, and the number of target joints (P<0.05) ; the improvement in the intermediate-dose prophylaxis group was better than that in the low-dose prophylaxis group (P<0.05). Compared with on-demand treatment, the FISH score, quality of life score, and SF-36 score significantly improved in both groups (P<0.05), but there was no significant difference between the two groups (P>0.05) . Conclusion: In Chinese adults with severe hemophilia A, low- and intermediate-dose prophylaxis can significantly reduce bleeding frequency, delay the progression of joint lesions, and improve the quality of life of patients as compared with on-demand treatment. The improvement in clinical bleeding was better with intermediate-dose prophylaxis than low-dose prophylaxis.
