Bleeding Symptoms And Laboratory Correlation In 150 Patients With Severe Von Willebrand Disease

Severe von Willebrand disease (VWD) is a rare bleeding disorder with markedly decreased or absent von Willebrand factor (VWF) protein, with a parallel decrease in VWF function and FVIII activity. Patients experience excessive mucosal bleeding, characteristic of VWD, but may also experience joint bleeding, a phenotype more typical of hemophilia A. The CDC sponsored Universal Data Collection (UDC) project collects joint range of motion (ROM) as well as other clinical data on patients with blood disorders followed at Hemophilia Treatment Centers (HTCs) in the U.S. As of 1/07, 253 patients in the UDC were classified as having severe VWD. The goal of this study was to further characterize these patients and assess relations between levels of FVIII/VWF and joint bleeding and function.