We are thankful to Eapen et al. for their interest in our work and for sharing their data with us. We reported in a large cohort that von Willebrand factor antigen (vWF-Ag) might be an easy to use marker to detect portal hypertension, and to predict decompensation and death in cirrhosis patients. As mentioned in our article, it is known that elevated vWF-Ag levels might be a consequence of a reduced clearance of vWF-Ag resulting from decreased expression or activity of ADAMTS13 (vWF-Ag cleaving protease) in patients with cirrhosis. A recent publication investigated the potential prognostic value of ADAMTS13 for mortality in liver cirrhosis, stating that ADAMTS13 activity may be a useful prognostic marker that is equal or superior to the Child-Pugh score and the Model for Endstage Liver Disease (MELD) score to predict not only the short-term prognosis but also the long-term survival of cirrhosis patients, with similar prognostic levels, compared to the vWF-Ag in our study. These findings suggest a prognostic value both for ADAMTS13 and ULvWF multimers also in our cohort.
However, ADAMTS13 assays have very high interassay variability, especially in the middle range, although newly developed kits promise improved quality. Both analysis of ULvWF multimers and ADAMTS13 are considerably more expensive compared to vWF-Ag analysis. Additionally, they are not available in standard laboratories and they take a long time to perform. This lack of clinical applicability led us to not analyze measurements of ULvWF or ADAMTS13 in our study.
MONIKA FERLITSCH, M.D. MARKUS PECK-RADOSAVLJEVIC, M.D. ARNULF FERLITSCH, M.D.
Vienna Hepatic Hemodynamic Laboratory, Internal Medicine III, Department of Gastroenterology & Hepatology, Medical University of Vienna, Vienna, Austria