Epidemiology of fractures in patients with haemophilia

Haemophilia is an X-chromosome linked inherited bleeding disorder characterised by an anomaly synthesis of coagulation factor VIII (Haemophilia A) or factor IX (Haemophilia B). There is very little information on the magnitude and management of fractures in PWH in the literature regards the advance on replacement therapy. The purpose of this paper is to present our 28 years experience treating PWH who suffered fractures and evaluate the impact of access to treatment.

In the period 1986-2013, 151 fractures in 141 PWH were treated, 125 patients type A (88.7%), 12 type B (8.5%) and 4 (2.8%) with von Willebrand's disease. For the sake of analysis we divided the fractures in five groups: 1986-1990: 25, 1991-1995: 35, 1996-2001: 33, 2002-2007: 31, and 2008-2013: 27; and classified the fractures in lower limb (LL) and upper limb (UL). We also considered the age at which the fractures occurred.

However the incidence of presentation of the fractures of the upper limb and lower limb changed through the years, being more frequent in the LL in the first period analysed (76% LL vs. 24% UL) and in the UL in the latter one (63% UL vs. 37% LL), the difference was statically significant (p=0.0168). In the relation with the age, the 1986-1990 cohort vs. 2008-2013 cohort reached statistical significance (p: 0.035). Regarding treatment, 121 fractures were treated in a non invasive way, the others 30 fractures, were treated with internal fixation. The patient treated with internal fixation had less mal-alignment, and delay consolidation.

This is the largest series of fractures in PWH published. We show a higher incidence of LL fractures in the first period analysed (1986-1990), over time, the ratio LL/UL changed as UL fractures became more frequent. This change is due to the access of the treatment and specifically to the prophylaxis.

Fractures in PWH have changed their pattern, becoming more common in the UL than in the LL, lowering the age at which they occur and being less frequent. We believe that the advent of new and accessibly treatments, decreased the development of orthopaedic complications and favours the improvement in quality of life of PWH.