Advances in treatment allow a near-normal lifestyle and lifespan for most patients with hemophilia A and B. Preservation of joint function may be achieved, even in patients with severe hemophilia A or B, with the use of prophylaxis (defined as the regular, continuous administration of a hemostatic agent/agents, with the goal of preventing bleeding in people with hemophilia while allowing them to lead active lives and achieve quality of life comparable to non-hemophilia individuals).[38]Srivastava A, Santagostino E, Dougall A, et al. WFH guidelines for the management of hemophilia, 3rd edition. Haemophilia. 2020 Aug;26 Suppl 6:1-158.
https://www.doi.org/10.1111/hae.14046
http://www.ncbi.nlm.nih.gov/pubmed/32744769?tool=bestpractice.com
Complications of hemophilia, such as chronic joint arthropathy, remain a common occurrence in developing countries, primarily in those who are not receiving prophylactic factor infusions or have developed inhibitors to factor VIII or factor IX. Several studies have shown prophylaxis with doses as low as 5 to 10 IU/kg 2 to 3 times a week substantially reduced the bleeding rate (compared with on-demand therapy), with improvement of quality of life allowing for better school attendance, gainful employment, and community participation.[141]Tang L, Wu R, Sun J, et al. Short-term low-dose secondary prophylaxis for severe/moderate haemophilia A children is beneficial to reduce bleed and improve daily activity, but there are obstacle in its execution: a multi-centre pilot study in China. Haemophilia. 2013 Jan;19(1):27-34.
https://onlinelibrary.wiley.com/doi/full/10.1111/j.1365-2516.2012.02926.x
http://www.ncbi.nlm.nih.gov/pubmed/23231016?tool=bestpractice.com
[142]Hua B, Lian X, Li K, et al. Low-dose tertiary prophylactic therapy reduces total number of bleeds and improves the ability to perform activities of daily living in adults with severe haemophilia A: a single-centre experience from Beijing. Blood Coagul Fibrinolysis. 2016 Mar;27(2):136-40.
http://www.ncbi.nlm.nih.gov/pubmed/26258677?tool=bestpractice.com
[143]Verma SP, Dutta TK, Mahadevan S, et al. A randomized study of very low-dose factor VIII prophylaxis in severe haemophilia: a success story from a resource limited country. Haemophilia. 2016 May;22(3):342-8.
http://www.ncbi.nlm.nih.gov/pubmed/26987935?tool=bestpractice.com
[144]Gouider E, Jouini L, Achour M, et al. Low dose prophylaxis in Tunisian children with haemophilia. Haemophilia. 2017 Jan;23(1):77-81.
http://www.ncbi.nlm.nih.gov/pubmed/27943510?tool=bestpractice.com
[145]Eshghi P, Sadeghi E, Tara SZ, et al. Iranian low-dose escalating prophylaxis regimen in children with severe hemophilia A and B. Clin Appl Thromb Hemost. 2018 Apr;24(3):513-8.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6714659
http://www.ncbi.nlm.nih.gov/pubmed/28049359?tool=bestpractice.com
Low-dose prophylaxis should therefore be advocated for patients in developing countries.