Von Willebrand disease is the most common inherited bleeding disorder, but it might be difficult to be diagnosed (1,2), indeed the total number of persons with von Willebrand disease reported in the last annual global survey is still lower than those reported with hemophilia, with a ratio of 0.4 (3)
In resource-limited settings, there are additional barriers to Von Willebrand disease diagnosis and management. Besides the lack of awareness in both public and healthcare provider (4), laboratory facilities and treatment are not available.
A need to optimize the use of the allocated budgets is mandatory.
In 2021, guidelines for the diagnosis and management of von Willebrand disease were published (5). These guidelines cannot be not applicable elsewhere and are even sometimes unknown (6).
Education and awareness, are a cornerstone. Collaboration between the patients' organizations and healthcare providers will enhance to improve awareness, screening and diagnosis of von Willebrand disease.
Healthcare providers building team and hemophilia treatment centre is another condition to improve the situation in low- and middle-income countries. The collaboration between clinicians and lab persons, is a pivotal need, to improve laboratory diagnosis. Laboratory assays and prioritization of the different von Willebrand assays with a referent laboratory is relevant.
Severe forms might be diagnosed easier, but frequently wrongly diagnosed as severe or moderate hemophilia A. Moderate and mild forms are often underdiagnosed.
Treatment will depend on the accurate diagnosis and the available therapeutic options.
Improvement of diagnosis will lead to better advocacy for a better treatment.
1- Rodeghiero F, Castaman G, Dini E. Epidemiological investigation of the prevalence of von Willebrand's disease. Blood. 1987 Feb;69(2):454-9. 2-Werner EJ, Broxson EH, Tucker EL, Giroux DS, Shults J, Abshire TC. Prevalence of von Willebrand disease in children: a multiethnic study. J Pediatr. 1993 ;123(6):893-8. 3- Report on the Annual Global Survey 2023, https://www1.wfh.org/publications/files/pdf-2525.pdf 4- O'Sullivan JM, Tootoonchian E, Ziemele B, Makris M, Federici AB, Khayat Djambas C, El Ekiaby M, Rotellini D, Sidonio RF, Iorio A, Coffin D, Pierce GF, Stonebraker J, James PD, Lavin M. Von Willebrand Disease: Gaining a global perspective. Haemophilia. 2023;29(4):1104-1112. 5- James PD, Connell NT, Ameer B, Di Paola J, Eikenboom J, Giraud N, Haberichter S, Jacobs-Pratt V, Konkle B, McLintock C, McRae S, R Montgomery R, O'Donnell JS, Scappe N, Sidonio R, Flood VH, Husainat N, Kalot MA, Mustafa RA. ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease. Blood Adv. 2021 Jan 12;5(1):280-300 6- Von Willebrand Disease in the East Mediterranean Region: Current Situation and Gaps C. Djambas Khayat1, E. Gouider Belhadjali2, M. El Khorassani3 and M. El-Ekiaby Volume 7 (Suppl. 2) October 2023, 101262
Learning Objectives:
In resource-limited setting, von Willebrand diagnosis and management strategies should be adapted to the context, and use of the ressources optimized. Awareness of both healthcare providers and general population is a cornerstone. Colloboration between clinicians and lab persons, will improve laboratory diagnosis. Therapeutic options will depend on the available treatments.
