Incidence, mortality rates and causes of deaths in haemophilia patients in Sweden

Abstract

Sweden has been a pioneer in the treatment of haemophilia, with the first concentrate available in the 1950s. Treatment has improved over the years to its current state-of-the art. The aim of the current study was to evaluate the long-term outcome of haemophilia in terms of incidence, morbidity and mortality. Patients diagnosed with haemophilia A or B registered at the national haemophilia centres and/or the Patient Registry and born before 2009 and alive in 1968 were enrolled and linked to the Cause of Death-, Migration- and Medical Birth registries. Five age- and sex-matched controls were selected for each patient. A total of 1431 patients with haemophilia A or B were compared with 7150 controls. The 3-year moving average incidence rate per 100,000 population varied between 21 and 36. The hazard ratio for all-cause mortality compared with controls was 2.2, 95% CI: [1.8; 2.7], P < 0.001 for the entire group of patients and 1.7, 95% CI: [1.3; 2.2], P < 0.001 when patients with HIV and/or viral hepatitis were excluded. The corresponding figures for the severe haemophilia subgroup were 6.6, 95% CI: [4.5; 10.0], P < 0.001 and 8.2, 95% CI [3.2; 20.8], P < 0.001 respectively. The most common causes of death were related to malignancies and the haemostatic defect. People with haemophilia were 57% less likely to die from ischaemic heart disease than controls. People with haemophilia in Sweden demonstrate higher mortality over time, independent of HIV and viral hepatitis, despite relatively advantageous access to clotting factor concentrates.

Total ankle replacement in patients with haemophilia and virus infections – a safe alternative to ankle arthrodesis?

“…With respect to the literature and to our own experience of a large haemophilia centre we believe that the patient population presented here constitutes a realistic cohort of haemophilic patients [34]. We are aware of the fact that the results of our study do not allow to draw any conclusions on the influence of virus infections in patients with haemophilia undergoing TAR.…”

Cardiovascular risk in patients with hemophilia

“…A more recent study comes from Sweden, where 1431 patients with hemophilia A or B were compared with 7150 controls by using a registry. 10 Thirteen percent of the hemophiliacs died from cardiovascular mortality compared with 29% of the controls. Mortality from stroke was not different between the 2 groups.…”

Bleeding‐related hospitalization in patients with von Willebrand disease and the impact of prophylaxis: Results from national registers in Sweden compared with normal controls and participants in the von Willebrand Disease Prophylaxis Network

“…Sweden has a long tradition of national registers containing high‐quality data including that kept by the National Board of Health and Welfare and Statistics, Sweden (SCB). National registers offer a view of the general population and enable studies with long‐term outcomes that can be examined retrospectively.…”