OBJECTIVES: Hemophilia A and B are disorders associated with the deficit of coagulation factors VIII and IX that increase the risk of bleeding and complications (clotting factor inhibitor antibodies, severe bleedings, hospitalizations and anaphylactic reactions) depending on the severity of the disorder. The objective of this study was to determine the incidence of complications in a cohort of patients diagnosed with moderate and severe hemophilia A or B under treatment in a specialized institution.

METHODS: A retrospective study of a cohort of patients with replacement therapy for hemophilia A or B, evaluating treatment and complications between January/2012 and July/2019. The characteristics associated to disease management, sociodemographic and clinical variables were extracted from the medical records. Time to inhibitor development and rate associated to bleeding and hospitalizations were evaluated.

RESULTS: A total of 159 male patients were identified with hemophilia A (n=140; 88.1%) and B (n=9; 11.9%) with a mean follow-up of 5.9±2.3 years. The mean age was 23.6±16.1 years and hemophilia was reported as severe in 125 patients (89.3%) and moderate in 13 (68.4%). Primary prophylaxis was registered in 17.0% of patients, 44.7% secondary, and 38.3% tertiary, mainly with recombinant factors (n=84; 52.8%) followed by plasma derived factors (n=75; 47.2%). The incidence of inhibitor development was 0.3 per 100 patients/year, with mean time to event of 509 days. The incidence of bleeding was 192 per 100 patients/year, especially at the joint (n=99; 62.3%) and muscle (n=25; 15.7%) level. The incidence of hospitalization was 3.7 per 100 patients/year and only 2 patients reported anaphylactic reactions to the factor.

CONCLUSIONS: The most common complication was joint bleeding which was expected in this type of patients. Low proportion of patients developed factor inhibitors during the follow up.