The frequency of iron deficiency among patients with haemophilia-A in northern Nigeria: correlation with the disease severity and clinical implications

Background Haemophilia-A (HA) is an X-linked recessive disorder characterized by the deficiency of functional clotting factor VIII resulting in lifelong bleeding diathesis. We predict that HA would be associated with iron deficiency and the risk will be higher in those with severe disease. If our prediction is correct, the frequency and the relative risk (RR) of iron deficiency will be higher in patients with severe HA in comparison with nonsevere HA. Materials and methods We evaluated the levels of haemoglobin concentrations, red cell indices and serum ferritin retrospectively with respect to the disease severity among a cohort of treatment-naive patients with HA as seen at the time of diagnosis in some hospitals in northern Nigeria. Results Out of the 39 patients studied, 19 were iron deficient, yielding an overall frequency of iron deficiency of 48.7%. Out of the 39 patients, 24 (61.5%) had severe HA and 15 (38.5%) had nonsevere HA. Patients with severe HA had a significantly higher frequency of iron deficiency in comparison with nonsevere HA (66.7 vs. 20%, P 0.004) and the RR of iron deficiency for patients with severe HA was 2.6 (95% confidence interval: 1.9–3.4, P = 0.003). Conclusion Iron deficiency is very common among patients with HA, the frequency and the RR of which is higher among patients with severe disease. Therefore, patients with HA should be regularly screened and treated for iron deficiency to prevent the adverse impact of iron deficiency on wound healing, the immunity and the mental development of haemophilic patients. However, the possibility of undesirable side effects of iron such as erosive gastritis, which can increase the risk of gastrointestinal haemorrhage in HA patients, calls for caution in the choice of oral pharmaceutical iron preparations visà- vis nonpharmaceutical dietary supplementation. Healthcare centres should formulate standard guidelines for the effective and safe treatment of iron deficiency in haemophilia patients.