Mortality and causes of death in Dutch haemophiliacs, 1973–86

Abstract

Summary. Mortality figures were calculated for a group of 717 Dutch haemophiliacs over the period 1973-86. Followup was on average 10-9 years; no patients were lost to followup. The data were compared to the general male population by actuarial methods and patient-year analysis. Forty-three patients died, while 20 deaths were expected in a hypothetical group of non-haemophiliacs of the same age distribution. Hence, overall mortality was 2·l times higher than in the general population. This leads to a calculated life expectancy of 66 years, äs compared to 74 years in the general male population. Mortality did not differ much by severity of haemophilia. A possibly beneflcial effect of prophylaxis on longevity was observed. Haemorrhage occurred in half of all deaths and among these traumatic bleeding was the most prevalent. The number of deaths due to ischaemic heart disease was significantly lower (80% reduction) than expected and therefore the authors conclude that haemophilia offers protection against ischaemic heart disease. Cancer mortality was significantly higher (2-5 times) than expected.

Prevalence and risk factors for heart disease among males with hemophilia

“…Several European studies [1][2][3] and one U.S. study [4] of cause-specific mortality have found lower rates of death from ischemic heart disease in persons with hemophilia compared to the general population. These findings have been interpreted as providing evidence that the hypocoagulable state offers protection from the thrombotic event that precipitates infarction.…”

Hepatitis C infection among Dutch haemophilia patients: a nationwide cross‐sectional study of prevalence and antiviral treatment

“…Since then nationwide surveys were repeated in 1978,1985,1992 and in 2001 [21][22][23][24]. These studies aimed at assessing the medical and social consequences of haemophilia in the Netherlands.…”

“…The mean age of patients differed according to severity of haemophilia and HCV status; patients with severe haemophilia, who were never infected, were younger [mean age 23 years, 95% confidence interval (CI) [19][20][21][22][23][24][25][26][27][28] …”

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

“…In 1986, thanks to the introduction of virally safe coagulation concentrates by the implementation of virucidal methods in the manufacturing process, individuals in the hemophilia community once again began to hope for a long, healthy, and active life. [6][7][8] Nowadays, given the improvement in coagulation concentrates, the life expectancy of individuals with hemophilia appears to be equal to that of individuals without hemophilia in developed countries. [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.…”