Survival of HIV Infected Individuals with Hemophilia after 20 Years: From the Canadian Hemophilia Registry.

Background: Patients with hemophilia A and B have been tracked by the Canadian Hemophilia Registry since 1980. The total number on the Registry is 3307 (A=2721, B=586) of which 662 were/are HIV-positive. New HIV and HCV infections ceased after 1985 and 1988 respectively with the introduction of safer blood products; HIV-infected individuals with hemophilia therefore resemble a cohort with HIV infection that was acquired in a relatively narrow time period about 20 years ago. Current status of HIV-positive patients is Active 250 (37.8%), Deceased 406 (61.3%), Lost to Follow Up 9 (0.9%). Follow up data is available for 3135/3307 (95% of all patients), and for 656/662 (99% of HIV-positive patients). We previously reported (

Objective: To update the rate and causes of death among HIV positive hemophilia patients in Canada with current information.

HIV-Related Mortality: The annual number of deaths in the HIV-positive group has been [year-#(deaths per 100 person-years)], 1980-0(0), 81-0(0),82-0(0),83-3(0.45),84-1(0.15),85-2(0.30),86-12(1.8),87-12(1.9),88-21(3.3),89-25(4.1),90-24(4.1),91-45(8.0),92-38(7.4),93-Currently, 256/662 (38.7%) are alive.

Causes of Death (HIV+): The commonest primary causes of death in HIV-positive individuals have been: [cause #(%)] AIDS 284(70), liver failure 45(11), bleeding 18(4.4) and infections 15(3.7). Twenty-eight patients had liver disease listed as a secondary or contributing cause of death; thus liver disease was the primary or contributory cause of death in 45+28=73 individuals (18% of HIV-positive deaths).

HCV-Related Deaths: HCV status is positive in 1151 of the total population of 3307 (35%) individuals in the registry, negative in 1260 (38%) and unknown in 896 (27%). Of HIV-positive individuals, 448 (68%) are co-infected with HCV, 53 of whom have died with liver failure (12% overall, 18% of deaths). HIV/HCV co-infected individuals have higher overall mortality than those not co-infected (46% vs. 12%, p<0.0001). In addition, HCV singly-infected individuals have lower overall mortality (7%). 703 HIV-negative individuals are infected with HCV, of whom 7 have died with liver failure (1% overall, 13% of deaths).

Conclusions: The death rate of HIV-positive individuals peaked in the years 1991-3 and decreased thereafter with 38% of individuals still living at 20 years follow up. However deaths still occur at an annual rate of 3–5%. HIV/HCV co-infected individuals have the highest mortality and HCV single-infected individuals have the lowest mortality of those with transfusion-transmitted infection. Liver failure is a common mode of death in both HIV- and HCV-infected individuals.