Laboratory Diagnosis and Classification of Von Willebrand Disease: A Review

Abstract

Von Willebrand Disease (VWD), as the most common inherited bleeding disorder, is a widely misdiagnosed disease due to several diagnostic pitfalls. At the heart of this challenge lies the complexity and heterogeneity of VWF, significant (pre-) analytic issues, limited access to a comprehensive repertoire of laboratory assays, inter-individual variations, lack of expertise and complex interpretation of results. Next to a personal and family bleeding history, an array of clinical laboratory tests is required because no single test reflects both quantity and quality of VWF. The assays measure different VWF properties and may be affected by (pre-) analytic variables possibly leading to inaccurate interpretation. Therefore, a laboratory investigation and diagnosis according to a standard algorithm, and repetitive testing, are essential for accurate diagnosis which is illustrated in detail in this review.