To evaluate the incidence and prevalence of hemophilia in Italy and th
e impact of HIV infection on the Italian hemophiliac population, data
from a computerized national registry of patients from 95% of the hemo
philia care centers in Italy were analyzed. A total of 4643 patients w
ere included in the registry. The prevalence of hemophilia A was 8.2 p
er 100,000 males, with no significant regional differences; for hemoph
ilia B the corresponding figure was 1.5 per 100,000. Temporal trends i
n hemophilia incidence suggest that the diagnosis of mild and moderate
hemophilia has improved. The overall HIV prevalence was 26% and was s
ignificantly (p < 0.001) higher in patients with hemophilia B (47.1%)
compared to those with hemophilia A (26.8%) or other diseases (16.5%).
The highest rate of HIV seropositivity was among patients 20-29 years
of age. The annual amount of clotting factor concentrates received wa
s significantly (p < 0.001) higher in HIV seropositive patients than i
n those who were seronegative. Antibody testing was never performed on
10.1% of severely affected patients. The number of patients in the It
alian registry was similar to the number that would have been expected
based on prevalence estimates from other countries. In comparison wit
h other countries, the prevalence of HIV infection recorded in Italy w
as lower in persons with hemophilia A, but higher in those with hemoph
ilia B. Our study demonstrates the usefulness of a registry in delinea
ting the epidemiology of hemophilia and in studying risk factors for H
IV infection. It also underlines the need for continuing surveillance
of this population.