Reported prevalence of von Willebrand disease worldwide in relation to income classification

Abstract

Introduction:The diagnosis of von Willebrand disease (VWD) is complex and challenging, especially when diagnostic resources are limited. This results in a lack of consistency in identifying and reporting the number of people with VWD and variations in the VWD prevalence worldwide. Aim:To analyze the reported prevalence of VWD worldwide in relation to income classification.Methods: Data on the VWD prevalence from the World Federation of Hemophilia Annual Global Survey, national registries of Australia, Canada, and the United Kingdom, and the literature were analysed. The income level of each country was classified according to the World Bank. Results:The mean VWD prevalence worldwide was 25.6 per million people. The VWD prevalence for high-income countries (HIC) of 60.3 per million people was significantly greater (p < .01) than upper middle (12.6), lower middle (2.5) and low (1.1) income countries. The type 3 VWD prevalence for HIC of 3.3 per million people was significantly greater (p < .01) than lower middle (1.3) and low income (0.7) countries. The reported VWD prevalence was greater among females than males. Conclusion:The reported VWD prevalence varied considerably across and within income classifications. The variability of type 3 VWD prevalence was less than the VWD prevalence (all types). The variability in detection and diagnosis of type 1 VWD presents a challenge in forming a consistent prevalence value across countries and income classifications.

Diagnosis and treatment challenges in lower resource countries: State‐of‐the‐art

“…In HIC, diagnostic delays for PwVWD are frequently encountered, particularly for females 28–30 . Despite the expected prevalence of 0.1%–1%, actual registration rates of PwVWD are much lower, with 2020 data identifying median registration rates in HICs of 45.1 per million population 31–34 . There is a considerable gap seen in lower and lower‐middle‐income countries (LIC/LMIC), with registration rates 100 times lower again (median 0.5–0.6/million population) 33 .…”

“…There is a considerable gap seen in lower and lower‐middle‐income countries (LIC/LMIC), with registration rates 100 times lower again (median 0.5–0.6/million population) 33 . The representation of VWD types is inverted in lower‐income settings, with type 2 and 3 VWDs dominating, compared to the majority of kind 1 VWD in HICs 33,34 . There remains a lack of data focused on VWD in LMIC/LICs, and we still need to adapt diagnostic and treatment algorithms to improve care in these settings.…”

“…Following the WOMAN study, tranexamic acid was incorporated, but only in its intravenous form 37,38 . Even in countries where oral tranexamic acid is widely available, medicine shortages have been reported 34 . These issues, in combination with the global lack of intranasal DDAVP, severely limit treatment options for PwVWD internationally 39 .…”

Laparoscopic liver resection for a patient of hepatocellular carcinoma with von Willebrand disease: a case report

“…However, the safety of LH in patients with inherited coagulation disorders (ICD), such as von Willebrand Disease (VWD), has not yet been demonstrated. VWD is the second most common ICD, with an estimated prevalence of 60.3 per million in high socioeconomic status countries [ 2 ]. Regarding the mechanism of the VWD, thrombocytes cannot adhere to the vessel walls due to von Willebrand factor (VWF) deficiency [ 3 ].…”