Primary prophylaxis was associated with lower arthropathy in Colombian men with haemophilia B: A longitudinal analysis (2015-2019)

Haemophilia. 2020 Nov;26(6):e282-e290. doi: 10.1111/hae.14136. Epub 2020 Sep 6.

Abstract

Introduction: The risk of chronic haemophilic arthropathy (CHA) is related to severity. Evidence suggests that primary prophylaxis (PPr) could reduce CHA incidence and its impact on quality of life.

Aim: To evaluate the association between PPr and CHA in Colombian males with haemophilia B (HB) during 2015 to 2019.

Methods: A panel-time analysis was performed with data provided by the National Health System to update a nationwide open cohort of people with congenital coagulopathies. The association was evaluated in a logistic random-effect regression model (LRERM), adjusted by age at diagnosis, prophylaxis dose and frequency, severity, haemarthrosis and high-titre inhibitors.

Results: During 2015-2019, a total of 362 men with HB and treated with either, primary, secondary or tertiary prophylaxis were identified. At baseline, CHA prevalence in the cohort was 36.84% (n = 133), median age was 19.0 years (IQR: 10.0-27.0), and median age at diagnosis was 1.0 year (IQR: 0.0-4.0). PPr was prescribed in 37.85% (n = 137), and median dose (IU/Kg/dose) was almost the same for primary vs. secondary/tertiary prophylaxis. Patients in PPr had a lower frequency of severe HB, CHA, haemarthrosis, infectious complications and high-titre inhibitors than those in secondary or tertiary prophylaxis (STPr). In the LRERM, PPr was associated with a significant reduction of 89.70% in the odds of CHA (aOR = 0.103, IC 95%: 0.040, 0.270; P < .001), compared with STPr.

Conclusions: PPr decreased the odds of CHA by 89.70% in males with HB in Colombia. Our findings are consistent with previous studies and support the strategy to prescribe PPr to our patients.

Keywords: haemophilia B; haemophilic arthropathy; men; prophylaxis; registries.

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Colombia
  • Hemophilia B / complications*
  • Hemophilia B / therapy*
  • Humans
  • Infant
  • Joint Diseases / etiology*
  • Longitudinal Studies
  • Male
  • Quality of Life / psychology*
  • Young Adult