Prevalence and severity of anaemia in persons with haemophilia and von Willebrand disease at Charlotte Maxeke Johannesburg Academic Hospital, South Africa

Background. Haemophilia and von Willebrand disease (VWD) are inherited bleeding diatheses characterised by spontaneous or traumatic bleeding resulting in varying degrees of anaemia. Early diagnosis, treatment and prevention of anaemia are crucial to improving physical and mental health and enhancing health-related quality of life. The global prevalence of anaemia and its associated risk factors is well established; however, there is a paucity of data on those with inherited bleeding disorders (IBD). Objectives. To describe the prevalence and severity of anaemia in haemophilia and VWD in a quaternary care facility. Methods. Adult patients with haemophilia or VWD of any subtype were identified through hospital record reviews. After excluding those without anaemia, defined as haemoglobin (HB) <13 g/dL for males and <12 g/dL for females, data from patients with anaemia were anonymised, captured, collated and analysed. Quantitative data were summarised with standard statistical tools, and qualitative data were described. The IBD cohort demographics, anaemia severity and prevalence data were compared with those of the controls, who were age- and sex-matched adult patients admitted to the haematology ward. Results. Of 1 100 patients with IBD screened, 77 met the eligibility criteria. These comprised 68 (88.3%) haemophilia patients and 9 (11.7%) patients with VWD. The majority of IBD patients were males, comprising 90.9% (n=70), while females comprised 9.1% (n=7). Of the 886 screened controls, 77 age- and sex-matched patients were selected for comparison. The prevalence of anaemia in the study cohort was 38.96% (n=30). Most patients with anaemia (96.7%, n=29) were male, with only a single female, while in the control group, 85.7% (n=66) were male and 14.3% (n=11) were female. The prevalence of severe anaemia, defined as HB <8 g/dL, was 7% in the IBD group, compared with 22.8% in the control group. In the IBD group, 40% (n=12) of patients had borderline anaemia, compared with the control population with a predominance of life-threatening anaemia (31.2%, n=24). Mild anaemia (HB <11 g/dL) was noted in 37% of the IBD study cohort v. 13% in the control population. Life-threatening anaemia was seen in 13% of the IBD cohort v. 31.2% in the control population. The prevalence of moderate anaemia was 3% in the IBD cohort v. 28.6% in controls. In the IBD cohort, 43% of the anaemic patients had iron deficiency anaemia, and 6.5% of patients in the control group had iron deficiency anaemia. Conclusion. This study indicates the burden of anaemia in the IBD population. Health professionals must be proactive in screening and treating anaemia in these patients. Further research is required to explore additional contributing factors. Optimisation of therapeutic strategies tailored to the unique needs of these patients is vital.