The best of times, the worst of times: a story of haemophilia

The History and Evolution of the Clinical Effectiveness of Haemophilia Type A Treatment: A Systematic Review

“…In 1940 the first successful medical treatment for haemophilia was published in the Lancet, an 11-year-old boy that experienced a major bleeding episode after a squint surgery was experimentally treated with a whole-blood transfusion and survived [2]. Further advances in transfusion technology achieved during World War II, eased access of haemophiliac patients to blood or plasma transfusions, as a result life expectancy reached in average 39.7 years [5].…”

“…Even though there was a boom in the rates of blood donations, the screening process was far from systematic and some donors were considered to be at greater risk of transmissible diseases than the general population. Infusion and transfusion of plasma derivatives were not safe and complications started to appear soon after [5].…”

Mixed Genotypes in Hepatitis C Virus Infection

“…Virtually, all hemophiliacs who received clotting factor concentrates prior to implementation of viral inactivation techniques became infected with hepatitis C virus at the time of the first infusion (Morfini et al, 1994;Lee C et al, 2002;Ragni et al, 2010). Prevalence rates of HCV infection up to 100% were reported in hemophilia patients treated with concentrates before 1985 (Yee et al, 2000;Lee C, 2009;Manucci, 2008;Arnold et al, 2006). Even though the introduction of heat-treated factor concentrates progressively decreased HCV transmission, the true risk ended when new regulations in blood donor screening together with the implementation of second and third generation immunoassays for the detection of antibodies against HCV was introduced in 1991 in Europe, in 1992 in the US and 1993 in Argentina (Morfini et al, 1994;Franchini et al, 2001;Lee and Dusheiko, 2002;Tagliaferri et al, 2010;Argentinean Ministry of Health resolution #1077, 1993.…”

“…Unfortunately, they also carried an increased risk of bloodborne viral infections, largely due to their preparation from pools of plasma collected from thousands of donors. Consequently, the use of clotting factor concentrates resulted in human immunodeficiency virus (HIV) and hepatitis C virus (HCV) epidemics in this population (Lee C, 1995(Lee C, , 2009Eyster, 2008;Ragni et al, 2010). As reported in different cohorts around the world, many patients with hemophilia became infected with HIV between 1982 and 1985.…”

“…The HCV epidemic was a much longer one, occurring between 1961 and 1985. The first patients became infected from the first large pool plasma-derived FIX concentrates and the epidemic ended with the dry heating of concentrates in 1985 (Lee C, 2009). Particularly in Argentina, commercial factor concentrates were not accessible until 1975.…”