MORTALITY IN PATIENTS WITH HEMOPHILIA - CHANGES IN A DUTCH POPULATIONFROM 1986 TO 1992 AND 1973 TO 1986

Objective: To determine causes of death and mortality rates in patient s with hemophilia over a period of 20 years, to assess changes in mort ality, and to distinguish between hemophilia-related death and recent death induced by viral infections. Design: Cohort study of 919 patient s followed from January 1986 to June 1992. Results were compared with outcomes of previous follow-up from 1973 to 1986. Setting: Consecutive national questionnaire surveys on hemophilia, using patient registrie s of the Netherlands Hemophilia Society and Dutch hemophilia centers. Patients: 919 males with hemophilia A or B who participated in a natio nal questionnaire survey on hemophilia in 1985. Median duration of fol low-up was 6.4 years, which yielded 5753 person-years of follow-up. Th e mean age at study entry was 30 years (range, 1 to 85 years). Measure ments: Standardized mortality ratios, causes of death, median life exp ectancy, age-adjusted relative risks associated with the type or sever ity of hemophilia, presence of inhibitors, prophylaxis, and human immu nodeficiency virus infection. Results: 45 patients (5%) died between J anuary 1986 and June 1992; 22.6 patients had been expected to die. Thu s, the overall standardized mortality ratio was 2.0. The overall media n life expectancy was 66 years for the cohort studied from 1973 to 198 6 and 68 years for the cohort studied from 1986 to 1992. When deaths r elated to viral infection were excluded, the life expectancy almost eq ualed that of the general male population. Between 1986 and 1992, 1 pa tient died of ischemic heart disease compared with the 5.2 who were ex pected to die of this disease. Infection with HIV was the strongest in dependent predictor of death (relative risk, 27.5 [95% CI, 5.7 to 132. 8]). After adjustment for HIV infection, no other hemophilia-related r isk factors were associated with the risk for death. Conclusions: The acquired immunodeficiency syndrome and hepatitis strongly influence mo rtality in patients with hemophilia. In the absence of viral infection s, the life expectancy of patients with hemophilia would almost equal that of the general male population.