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Mortality and causes of death in patients with hemophilia, 1992-2001: a prospective cohort study.
J Thromb Haemost. 2006 Mar; 4(3):510-6.JT

Abstract

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.

Authors+Show Affiliations

Plug I
Leiden University Medical Center, Clinical Epidemiology, Leiden, the Netherlands.
Van Der Bom JG
No affiliation info available
Peters M
No affiliation info available
Mauser-Bunschoten EP
No affiliation info available
De Goede-Bolder A
No affiliation info available
Heijnen L
No affiliation info available
Smit C
No affiliation info available
Willemse J
No affiliation info available
Rosendaal FR
No affiliation info available

MeSH

AdolescentAdultAgedAged, 80 and overCause of DeathChildChild, PreschoolHIV InfectionsHemophilia AHepatitis CHumansInfantInfant, NewbornLife ExpectancyMaleMiddle AgedNetherlandsProspective StudiesSeverity of Illness Index

Pub Type(s)

Comparative Study
Journal Article

Language

eng

PubMed ID

16460432

Clinical Trial Links

Prospective Biomarkers of Bone Metabolism in Hemophilia A

Citation

Plug, I, et al. "Mortality and Causes of Death in Patients With Hemophilia, 1992-2001: a Prospective Cohort Study." Journal of Thrombosis and Haemostasis : JTH, vol. 4, no. 3, 2006, pp. 510-6.
Plug I, Van Der Bom JG, Peters M, et al. Mortality and causes of death in patients with hemophilia, 1992-2001: a prospective cohort study. J Thromb Haemost. 2006;4(3):510-6.
Plug, I., Van Der Bom, J. G., Peters, M., Mauser-Bunschoten, E. P., De Goede-Bolder, A., Heijnen, L., Smit, C., Willemse, J., & Rosendaal, F. R. (2006). Mortality and causes of death in patients with hemophilia, 1992-2001: a prospective cohort study. Journal of Thrombosis and Haemostasis : JTH, 4(3), 510-6.
Plug I, et al. Mortality and Causes of Death in Patients With Hemophilia, 1992-2001: a Prospective Cohort Study. J Thromb Haemost. 2006;4(3):510-6. PubMed PMID: 16460432.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Mortality and causes of death in patients with hemophilia, 1992-2001: a prospective cohort study. AU - Plug,I, AU - Van Der Bom,J G, AU - Peters,M, AU - Mauser-Bunschoten,E P, AU - De Goede-Bolder,A, AU - Heijnen,L, AU - Smit,C, AU - Willemse,J, AU - Rosendaal,F R, PY - 2006/2/8/pubmed PY - 2006/5/25/medline PY - 2006/2/8/entrez SP - 510 EP - 6 JF - Journal of thrombosis and haemostasis : JTH JO - J Thromb Haemost VL - 4 IS - 3 N2 - 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. SN - 1538-7933 UR - https://www.unboundmedicine.com/medline/citation/16460432/Mortality_and_causes_of_death_in_patients_with_hemophilia_1992_2001:_a_prospective_cohort_study_ DB - PRIME DP - Unbound Medicine ER -