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
Leiden University Medical Center, Clinical Epidemiology, Leiden, the Netherlands.
J Thromb Haemost. 2006 Mar;4(3):510-6. doi: 10.1111/j.1538-7836.2006.01808.x.
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.
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.
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).
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.
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.
自1985年起，凝血因子产品对艾滋病毒而言是安全的，自1992年起对丙型肝炎也是安全的。很少有研究报告病毒感染传播风险期过后血友病患者总人群的死亡率情况。
我们研究了1992年至2001年间血友病患者的死亡率、死亡原因和预期寿命。我们将这些结果与之前几个队列的结果进行了比较，这些队列涵盖了使用潜在受污染凝血产品之前、期间和之后的时期。
我们对967例甲型和乙型血友病患者进行了一项前瞻性队列研究。将总体和特定病因的死亡率与按年龄和日历期调整的荷兰男性全国死亡率数据进行比较，作为标准化死亡率（SMR）。
1992年至2001年间，94例（9.7%）患者死亡，2例患者失访（0.2%）。血友病患者的死亡率比一般男性人群高2.3倍（SMR 2.3，95%置信区间1.9 - 2.8）。在重度血友病患者中，预期寿命从63岁（1972 - 1985年）降至59岁（1992 - 2001年）。排除与病毒相关的死亡后，出生时的预期寿命为72岁。
艾滋病是主要死亡原因（26%），22%的死亡是由丙型肝炎导致的。在未受病毒感染的患者中，与荷兰男性人群相比，死亡率似乎仍有适度上升的趋势。因此，血友病患者的死亡率仍然较高；这在很大程度上是病毒感染的后果。
