Prevalence and Severity of Anaemia in Persons with Haemophilia and Von Willebrand Disease at Charlotte Maxeke Johannesburg Academic Hospital

Abstract

Background: Haemophilia and von Willebrand Disease (vWD) are inherited bleeding diathesis characterized 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 associated risk factors are well established, however, there is paucity of data on those with inherited bleeding disorders (IBD). The objective of this study was to describe the prevalence and severity of anaemia in haemophilia and vWD in a quaternary care facility. Methods: This study was approved by the institutional ethics and facility authorities. Adult patients with haemophilia or vWD of any subtype were identified through the hospital record reviews. After excluding those who did not fulfil the inclusion criteria, data from the selected patients was anonymised, captured, collated and analysed. Quantitative data was summarised with standard statistical tools and qualitative data was described. The IBD cohort demographics, anaemia severity and prevalence data was compared to those of the controls. Results: Of the 1 100 IBD patients screened, 77 met the eligibility criteria. This comprised of 68 (88,3%) haemophilia patients and 9 (11.7%) patients with VWD. The majority of IBD patients were males, accounting for 90.9% (n=70), while females comprised 9.1% (n=7). Of the 886 screened controls, 77 age and sex-matched were selected for comparison. This comprised 85.7% (n=66) male patients and 14.3% (n=11) female patients. The prevalence of anaemia in the IBD cohort was 38,96% (n=30); of which 96.7% (n=29) were male and only a single female. The majority (40%, n=12) of the anaemic IBD patients had a borderline anaemia compared to the control population which displayed a predominance of life-threatening anaemia (31,2%, n=24). Mild anaemia (HB <11g/dL) was noted in 37% of the anaemic IBD study cohort vs 13% in the control population. The prevalence of moderate anaemia was 3% in the IBD cohort vs 28,6% in controls. Life-threatening anaemia, was seen in 13% of the anaemic IBD cohort vs 31,2% in the control population. 43% of the anaemic patients had a documented iron deficiency anaemia while 6.5% control patients had an iron deficiency. Conclusion: This study indicates the burden of anaemia in the IBD population. Thus health professionals must be proactive in screening and treating their anaemia. Further research is required to explore additional contributing factors. Optimization of therapeutic strategies tailored to the unique needs of these patients is vital.