Mortality Trends and Causes of Death in Persons with Hemophilia in the United States, 1999-2014

ASH 2017 (Atlanta, GA, December 9-12): Historically, the most common cause of death among persons with hemophilia (PWH) in the United States (US) was related to bleeding events, and the median age at death was around 25 years; however in recent years death as a result pf bleeding has declined. Hemophilia-related deaths were examined using the 1999-2014 US multiple cause-of-death mortality data. Hemophilia deaths were identified as deaths for which a diagnosis code for hemophilia was listed anywhere on the death record.

From 1999-2014 there were 2,354 hemophilia-related deaths reported in the US. The hemophilia-related death rate decreased from 0.15 hemophilia-related deaths per 100,000 population to 0.08 hemophilia-related deaths per 100,000 population. The median age at death increased from 49 years in 1999 to 63 years in 2014.  From 1999-2002, HIV was most commonly listed as an underlying or contributing cause of death, while chronic cardiac complications was most commonly listed as the underlying or contributing cause of death from 2011-2014.The most common underlying or contributing cause of death also differed by age group. Among deaths in persons <20 years of age, bleeding into the brain (intracranial hemorrhage) was the most common. The most common underlying or contributing causes of death among deaths occurring between 20 and 69 years of age were HIV and/or hepatitis. The most common underlying or contributing cause of death among deaths occurring at 70+ years of age was chronic cardiac complications. Compared to non-hemophilia-related deaths, deaths related to hemophilia were more likely to be related to HIV, hepatitis, hemorrhage, and specifically intracranial hemorrhage. Interestingly, hemophilia-related deaths were less likely to be related to cardiac complications and cancer than non-hemophilia-related deaths.

This report highlights the continued success of interventions to decrease death among PWH. However, this report also highlights possible areas of future research in hemophilia, including monitoring trends in morbidities related to aging, such as cardiac disease and comorbidities due to chronic hepatitis infection.