Correlation between environmental factors and severity of bleeding in hemophilia A

doi:10.3969/j.issn.1006-7795.2019.01.021

Abstract

Abstract: Objective To observe the correlation between the environmental factors of clinical heterogeneity and the severity of bleeding in patients of severe hemophilia A with intron 22 (Inv22) and intron 1 (Inv1) inversion. Methods A retrospective analysis of 63 patients of severe hemophilia A with Inv22 and Inv1 who were enrolled in the Shanxi Hemophilia Management Center from 2009 to 2012 was conducted.The general information, first bleeding age, first joint bleeding age, first treatment age, annualized bleed rate, were recorded in detail. One-way analysis and Logistic regressive were used to analysis the correlation between environmental factors and severity of bleeding. Results Among the 63 patients, the average age was (17.75±8.51) years, the median age was 15 years, the average annual bleeding rate was (77.83±56.43) times per year, and the median annualized bleeding rate was 60 times per year. The age, the first treatment age, and treatment regimen were statistically significant(P<0.05)within univariate analysis. Ordered Logistic regression analysis results showed that patients with first joint bleeding age ≤ 1 years were more likely to suffer from bleeding (OR=13.97, 95% CI:2.32-84.52), the first treatment of patients aged ≤ 1 year was less likely to suffer from bleeding (OR=0.03, 95% CI:0.01-0.49). Conclusion Among the patients of severe hemophilia A with Inv22 and Inv1, patients with the first joint bleeding age of ≤ 1 years were more likely to suffer from bleeding, and patients with age ≤ 1 years were less likely to suffer from bleeding. The first joint bleeding age can be used as an indicator of the severity of bleeding. It is recommended to give an earlier prophylaxis to avoid the occurrence of severe bleeding later.

Key words: hemophilia A, intron inversion, clinical heterogeneity, environmental factors

CLC Number:

Chen Kun, Yang Linhua, Wang Gang, Liu Xiuer, Qin Xiuyu, Zhang Xialin. Correlation between environmental factors and severity of bleeding in hemophilia A[J]. Journal of Capital Medical University, 2019, 40(1): 119-123.

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URL: https://journal03.magtech.org.cn/Jweb_sdykdxxb/EN/10.3969/j.issn.1006-7795.2019.01.021

https://journal03.magtech.org.cn/Jweb_sdykdxxb/EN/Y2019/V40/I1/119

References

[1] 中华医学会血液学分会血栓与止血学组、中国血友病协作组. 血友病诊断与治疗中国专家共识(2017年版)[J]. 中华血液学杂志, 2016,38(5):364-370.
[2] Astermark,J, Petrini P, Tengborn L, et al. Primary prophylaxis in severe haemophilia should be started at an early age but can be individualized[J].Br J Haematol,2015,105(4):1109-1113.
[3] Pavlova A, Oldenburg J. Defining severity of hemophilia:more than factor levels[J]. Semin Thromb Hemost, 2013, 39(7):702-710.
[4] Franchini M, Mannucci P M. Modifiers of clinical phenotype in severe congenital hemophilia[J]. Thromb Res, 2017, 156:60-64.
[5] 樊萌语,吕筠,何平平. 国际体力活动问卷中体力活动水平的计算方法[J].中华流行病学杂志,2014,35(8):961-964.
[6] 李含, 孙竞. 体力活动状况对中国重型血友病A患者出血频率影响的观察[J]. 血栓与止血学, 2012, 18(3):127-130.
[7] Lannoy N, Ravoet M, Grisart B, et al. Five int22h homologous copies at the Xq28, locus identified in intron22 inversion type 3 of the factor VⅢ,gene[J]. Thromb Res,2016,137:224-227.
[8] Hamasaki-Katagiri N,Lin B C,Simon J,et al. The importance of mRNA structure in determining the pathogenicity of synonymous and non-synonymous mutations in haemophilia[J]. Haemophilia,2017,23(1):e8-e17.
[9] Santagostino E, Mancuso M E, Tripodi A, et al. Severe hemophilia with mild bleeding phenotype:molecular characterization and global coagulation profile[J]. J Thromb Haemost, 2010, 8(4):737-743.
[10] van den Berg H M, De Groot P H, Fischer K. Phenotypic heterogeneity in severe hemophilia[J]. J Thromb Haemost, 2007, 5(Suppl 1):151-156.
[11] van Dijk K, Fischer K, van der Bom J G, et al. Variability in clinical phenotype of severe haemophilia:the role of the first joint bleed[J]. Haemophilia, 2010, 11(5):438-443.
[12] 李含, 孙竞, 周璇,等. 中国重型血友病A的临床表现异质性及早期预测指标[J]. 南方医科大学学报, 2013, 33(3):424-427.
[13] Richards M, Lavigne Lissalde G, Combescure C, et al. Neonatal bleeding in haemophilia:a European cohort study[J]. Br J Haematol, 2012, 156(3):374-382.
[14] Biere-Rafi S, Haak B W, Peters M, et al. The impairment in daily life of obese haemophiliacs[J]. Haemophilia, 2015, 17(2):204-208.
[15] Tiktinsky R, Falk B, Heim M, et al. The effect of resistance training on the frequency of bleeding in haemophilia patients:a pilot study[J]. Haemophilia, 2002, 8(1):22-27.
[16] Tiktinsky R, Kenet G, Dvir Z, et al. Physical activity participation and bleeding characteristics in young patients with severe haemophilia[J]. Haemophilia, 2009, 15(3):695-700.