Real-world comparative analysis of bleeding complications and health-related quality of life in patients with haemophilia A and haemophilia B

Haemophilia. 2018 Sep;24(5):e322-e327. doi: 10.1111/hae.13596. Epub 2018 Aug 9.

Abstract

Introduction: Clinical severity and impact of haemophilia on quality of life have been generally considered to be lower for haemophilia B (HB) compared with haemophilia A (HA) patients.

Aims: To compare annual bleeding rate (ABR), target joint development and health-related quality of life (HRQoL) between adult (≥18 years) severe HA and HB patients using recent data from the Cost of Haemophilia in Europe: a Socioeconomic Survey (CHESS) study.

Methods: Multivariate generalized linear models (GLM) were constructed to assess the relationship between haemophilia type, ABR, HRQoL (derived from EQ-5D index scores) and the presence of target joints while controlling for covariates.

Results: Of the 1225 patients included, 77% (n = 949) had HA and 23% (n = 278) had HB. Of the 514 patients who completed the EQ-5D, 78% (n = 405) had HA, and 22% (n = 110) had HB. Unadjusted mean ABR was 3.79 in HA and 4.60 in HB. The presence of ≥1 target joint was reported in 59% and 54% of patients with HA and HB, respectively. Unadjusted mean EQ-5D index score was 0.78 in HA and 0.76 in HB. Haemophilia type was not a significant predictor of ABR, target joints or HRQoL when adjusted for confounding factors such as BMI, age and replacement therapy regimen.

Conclusion: Data suggest comparable ABR, incidence of target joints and HRQoL between patients with HB and HA indicating comparable clinical severity and disease impact on patient quality of life.

Keywords: annual bleed rate; haemophilia type (A & B); target joints, health-related quality of life.

MeSH terms

  • Adult
  • Cross-Sectional Studies
  • Female
  • Hemophilia A / complications*
  • Hemophilia A / pathology
  • Hemophilia B / complications*
  • Hemophilia B / pathology
  • Hemorrhage / etiology*
  • Hemorrhage / pathology
  • Humans
  • Male
  • Quality of Life / psychology*
  • Retrospective Studies

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