Beyond the guidelines: how we approach challenging scenarios in the diagnosis and management of von Willebrand disease

Although von Willebrand disease (VWD) is the most common inherited bleeding disorder, its diagnosis and management are often challenging. Clinical practice guidelines, developed through systematic review of the medical literature and considering the best available evidence, provide guidance for common clinical scenarios. However, in the clinical setting, patients often present with characteristics and nuances that may fall outside the realm of available evidence and guidelines, and hence, shared decision-making will be essential in the evaluation and management of these patients. The challenges in the diagnosis of VWD are mainly attributable to the heterogeneity of the disorder, limitations of laboratory assays, and the significant impact of various physiologic processes on von Willebrand factor. The impact of physiologic normalization of von Willebrand factor, which may occur in various settings such as pregnancy, inflammation, or aging, remains uncertain, as is the optimal management in these scenarios. Multidisciplinary and individualized care, based on evolving evidence supported by clinicians, patients, caregivers, and stakeholders, will be needed to ensure the highest quality care for those who live with VWD. •The heterogeneity of von Willebrand disease (VWD) often makes its diagnosis a challenge.•Available assays have limitations and are often significantly influenced by physiologic factors.•Physiologic normalization of von Willebrand factor in those with VWD poses a management dilemma.•Multidisciplinary, individualized care is essential, and several areas require further research.