Quarterly Journal of Medicine, 1994; 87:181-186 Association of severe haemophilia A with osteoporosis: a densitometric and biochemical study

Following a femoral neck fracture and vertebral compression fractures in two patients with severe haemophilia A, bone density and turnover were measured in 19 males with severe haemophilia A (all HIV negative, 18/19 hepatitis C antibody posit-ive) and in 19 age/sex matched controls. Bone den-sity at the lumbar spine (L2-4), measured by dual energy X-ray absorptiometry, was significantly lower in the haemophiliac patients (HPs) at (mean + SEM) 1.109 + 0.042 g/cm2 vs. 1.234 ±0.027 in controls; p = 0.018. Femoral neck density was also lower at 0.877 + 0.034 g/cm2 (HPs) vs. 1.067 ±0.032; p< 0.0005. No significant differences were evident between the groups for serum calcium, parathyroid hormone, luteinizing hormone, follicle-stimulating hormone or 1,25 dihydroxyvitamin D3, nor for fast-ing urinary hydroxyproline, pyridinoline or deoxy-pyridinoline excretion. Serum total alkaline phos-phatase was elevated in HPs at 200 ± 1 0 U/l vs. 158 + 8; p=0.004. Similarly, y-glutamyl transferase was elevated at 42 ± 7 U/l (HPs) vs. 20 ± 2; p = 0.007. Serum total testosterone and sex-hormone-binding globulin (SHBG) were higher in HPs at 26±2.5nmol / l vs. 17.4 ±1.6 (p = 0.009) and 56±6nmol / l vs. 27 ± 3 (p = 0.0005), respectively. Free androgen index, however, was lower in HPs at 44 ± 5 vs 69 ± 7; p = 0.008. These results suggest significant osteopenia associated with haemophilia A. This may be partly due to liver dys-function in HPs, but other factors, e.g. relative immobilization, may also be relevant