Major Changes of von Willebrand Factor Multimer Distribution in Cirrhotic Patients with Stable Disease or Acute Decompensation

Eszter Palyu; Jolan Harsfalvi; Tamas Tornai; Maria Papp; Miklos Udvardy; Katalin Szekeres-Csiki; Lajos Pataki; Karen Vanhoorelbeke; Hendrik B. Feys; Hans Deckmyn; Istvan Tornai

doi:10.1055/s-0038-1661393

Thrombosis and Haemostasis, Table of Contents

CC BY-NC-ND 4.0 · Thromb Haemost 2018; 118(08): 1397-1408
DOI: 10.1055/s-0038-1661393

Cellular Haemostasis and Platelets

Georg Thieme Verlag KG Stuttgart · New York

Major Changes of von Willebrand Factor Multimer Distribution in Cirrhotic Patients with Stable Disease or Acute Decompensation

Authors

Eszter Palyu^*

¹Division of Gastroenterology, Department of Internal Medicine, Faculty of Medicine, University of Debrecen, Debrecen, Hungary
Jolan Harsfalvi^*

²Department of Biophysics and Radiation Biology, Semmelweis University, Budapest, Hungary
Tamas Tornai

¹Division of Gastroenterology, Department of Internal Medicine, Faculty of Medicine, University of Debrecen, Debrecen, Hungary
Maria Papp

¹Division of Gastroenterology, Department of Internal Medicine, Faculty of Medicine, University of Debrecen, Debrecen, Hungary
Miklos Udvardy

³Department of Laboratory Medicine, Faculty of Medicine, University of Debrecen, Debrecen, Hungary
Katalin Szekeres-Csiki

³Department of Laboratory Medicine, Faculty of Medicine, University of Debrecen, Debrecen, Hungary
Lajos Pataki

³Department of Laboratory Medicine, Faculty of Medicine, University of Debrecen, Debrecen, Hungary
Karen Vanhoorelbeke

⁴Laboratory for Thrombosis Research, IRF Life Science, KU Leuven Kulak, Kortrijk, Belgium
Hendrik B. Feys

⁵Transfusion Research Center, Belgian Red Cross-Flanders, Ghent, Belgium

⁶Faculty of Medicine and Health Sciences, Ghent University, Ghent, Belgium
Hans Deckmyn

⁴Laboratory for Thrombosis Research, IRF Life Science, KU Leuven Kulak, Kortrijk, Belgium
Istvan Tornai

¹Division of Gastroenterology, Department of Internal Medicine, Faculty of Medicine, University of Debrecen, Debrecen, Hungary

Abstract

Background There is an unstable balance between pro- and anti-haemostatic processes in patients with cirrhosis. We hypothesized, that in patients with acute decompensation (AD) the major alterations of von Willebrand factor (VWF) could contribute to the pro-thrombotic situation as compared to patients with stable (ST) cirrhosis.

Patients and Methods We analysed different parameters of VWF, including detailed multimer distribution by densitometry and platelet adhesion, together with a disintegrin-like and metalloproteinase with thrombospondin type-1 motifs 13 (ADAMTS13) activity and antigen and C-reactive protein (CRP) levels in patients with ST cirrhosis (n = 99), with AD (n = 54) and controls (n = 92).

Results VWF antigen, ristocetin co-factor as well as collagen-binding activities were elevated in both cirrhotic groups in a stepwise manner. There was a decrease in high and an increase in low molecular weight multimer ratios in the majority of ST cirrhosis. However, in 24 out of 54 AD patients, ultra-large VWF multimers (ultra-large molecular weight multimers [ULMWM]) were found. ADAMTS13 activity in ST and AD patients without ULMWM was similar to controls (median [interquartile range; IQR]%: 98 [67–132] and 91 [60–110] vs. 106 [88–117], respectively). The presence of ULMWM in AD patients was associated with low ADAMTS13 activity [33 (24–49)%] and high CRP level [23 (7.1–83.6) mg/L]. Adhesion of normal platelets showed a stepwise increase in the presence of cirrhotic plasmas, reaching the highest level in AD patients with ULMWM.

Conclusion Characteristic changes of VWF parameters are seen in ST cirrhosis. In AD patients, highly increased VWF and reduced ADAMTS13 activity could be found, along with the presence of ULMWM, which are possible markers and contributors of the disease progression.

Keywords

cirrhosis - acute decompensation - systemic inflammation - von Willebrand factor multimers - thrombotic micro-angiopathy

Full Text

References

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