Joint health and treatment modalities in Nordic patients with moderate haemophilia A and B - The MoHem study.

Abstract

The prevalence of arthropathy in moderate haemophilia A (MHA) and B (MHB) is not well known. We evaluated joint health in Nordic patients in relation to their treatment modality. A cross-sectional, multicentre study covering MHA and MHB in Sweden, Finland and Norway. Arthropathy was evaluated by ultrasound (HEAD-US) and Haemophilia Joint Health Score (HJHS). We report on 145 patients: median age 28years (IQR 13-52) and 61% MHA. Baseline factor VIII/factor IX activity (FVIII/FIX:C) was 2IU/dL (median) (IQR 2-4): lower for MHB (2IU/dL, IQR 1-2) than MHA (3IU/dL, IQR 2-4) (P<.01). Eighty-five per cent of MHA and 73% MHB had a history of haemarthrosis (P=.07). Age at first joint bleed was lower for MHA (5years [median], IQR 3-7) than MHB (7years, IQR 5-12) (P=.01). Thirty-eight per cent received prophylaxis, started at median 10years of age (IQR 4-24). Median joint bleeds and serious other bleeds during the last 12months were both zero (IQR 0-1). Total HEAD-US captured 0/48 points (median) (IQR 0-2) and HJHS 4/120 points (IQR 1-10) with strong correlation between them (r=.72). C≤3IU/dL was associated with higher HJHS (P=.04). Fifteen per cent had undergone orthopaedic surgery. The current joint health in Nordic moderate haemophilia patients was rather good, but a subgroup had severe arthropathy. C≤3IU/dL and MHA were associated with a more severe bleeding phenotype. We suggest primary prophylaxis to all patients with FVIII/FIX:C≤3IU/dL.