Mortality and causes of death in patients with hemophilia, 1992–2001: a prospective cohort study

Abstract

Summary. Background: Clotting factor products have been safe for HIV since 1985, and for hepatitis C since 1992. Few studies have reported on mortality in the total population of hemophilia patients after the period of risk of viral infection transmission. Objectives: We studied the mortality, causes of death, and life expectancy of hemophilia patients between 1992 and 2001. We compared these findings with those of previous cohorts, together spanning the periods before, during, and after the use of potentially contaminated clotting products. Patients and methods: We performed a prospective cohort study among 967 patients with hemophilia A and B. Death rates, overall and cause‐specific, were compared with national mortality figures for males adjusted for age and calendar period as standardized mortality ratio (SMRs). Results: Between 1992 and 2001, 94 (9.7%) patients had died and two patients were lost to follow‐up (0.2%). Mortality was 2.3‐times higher in hemophilia patients than in the general male population (SMR 2.3 95% confidence interval 1.9–2.8). In patients with severe hemophilia, life expectancy decreased from 63 (1972–1985) to 59 years (1992–2001). Exclusion of virus‐related deaths resulted in a life expectancy at birth of 72 years. Conclusions: AIDS was the main cause of death (26%) and 22% of deaths were because of hepatitis C. In patients not affected by viral infections, there still appeared to be a trend toward a moderately increased mortality compared with the Dutch male population. Thus, mortality of patients with hemophilia is still increased; this is largely because of the consequences of viral infections.

Ageing successfully with haemophilia: A multidisciplinary programme

“…[1][2][3] In this context, more and more of them are becoming confronted with a new challenge: ageing. Ageing influences their well-being, because older PWH carry the burden of the physical and psychological consequences of the inadequate treatment received in the early periods of their lives.…”

Causes of Death Among 379 Patients With Hemophilia: A Developing Country’s Report

“…[9][10][11] Furthermore, recent findings have demonstrated that prophylaxis regimens decrease bleeding, pain, the frequency and duration of hospitalizations, disability, and quality of life in the hemophiliac population. Several groups of researchers 12,13 have hypothesized that there is an obvious link between the economic capacity of a country and the availability of the coagulation factors, diagnostic facilities, patients, care levels, and death rates due to bleeding disorders. These parameters can influence the difference in the rate and type of deaths among patients with hemophilia in various countries.…”

Characteristics of Older Patient with Haemophilia

“…They are usually associated with HCV and HIV positivity. Hepatocellular carcinoma is very important cause of death among haemophiliacs with a reported standardized mortality ratio of 17.2 (Plug, 2006) and 13.51 (Darby, 2007). Risk factors for hepatocellular carcinoma assessed in multicentre Italian study were presence of liver cirrhosis, elevated alpha fetoprotein, current HCV infection and age over 45 (Tradati, 1998).…”

“…Additionally, HIV infection and cART may per se increase the risk for metabolic and cardiovascular disease, as it was stated earlier. Indeed, it was reported recently an increasing num b e r o f d e a t h s i n haemophilia patients as a consequence of ischemic heart disease (Plug, 2006), together with increasing prevalence of ischemic heart disease among haemophilia patients 60 years of age and older (Kulkarni, 2005). Moreover, it is important to educate haemophilia patients about the cardiovascular risk factors, and on the benefit of living a healthy lifestyle ).…”