Advanced search
1 file | 646.93 KB Add to list

Major changes of von Willebrand factor multimer distribution in cirrhotic patients with stable disease or acute decompensation

(2018) THROMBOSIS AND HAEMOSTASIS. 118(8). p.1397-1408
Author
Organization
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, THROMBOTIC THROMBOCYTOPENIC PURPURA, ORTHOTOPIC LIVER-TRANSPLANTATION, FACTOR-CLEAVING PROTEASE, HEPATIC STELLATE CELLS, PLATELET ACTIVATION, HEPATOCELLULAR-CARCINOMA, BACTERIAL-INFECTIONS, VONWILLEBRAND-FACTOR, ADAMTS13, HEMOSTASIS

Downloads

  • major changes of VWF multimer distribution in cirrhotic patients with stable disease or acute decompensation, palyu, T&H.pdf
    • full text
    • |
    • open access
    • |
    • PDF
    • |
    • 646.93 KB

Citation

Please use this url to cite or link to this publication:

MLA
Palyu, Eszter, Jolan Harsfalvi, Tamas Tornai, et al. “Major Changes of Von Willebrand Factor Multimer Distribution in Cirrhotic Patients with Stable Disease or Acute Decompensation.” THROMBOSIS AND HAEMOSTASIS 118.8 (2018): 1397–1408. Print.
APA
Palyu, E., Harsfalvi, J., Tornai, T., Papp, M., Udvardy, M., Szekeres-Csiki, K., Pataki, L., et al. (2018). Major changes of von Willebrand factor multimer distribution in cirrhotic patients with stable disease or acute decompensation. THROMBOSIS AND HAEMOSTASIS, 118(8), 1397–1408.
Chicago author-date
Palyu, Eszter, Jolan Harsfalvi, Tamas Tornai, Maria Papp, Miklos Udvardy, Katalin Szekeres-Csiki, Lajos Pataki, et al. 2018. “Major Changes of Von Willebrand Factor Multimer Distribution in Cirrhotic Patients with Stable Disease or Acute Decompensation.” Thrombosis and Haemostasis 118 (8): 1397–1408.
Chicago author-date (all authors)
Palyu, Eszter, Jolan Harsfalvi, Tamas Tornai, Maria Papp, Miklos Udvardy, Katalin Szekeres-Csiki, Lajos Pataki, Karen Vanhoorelbeke, Hendrik Feys, Hans Deckmyn, and Istvan Tornai. 2018. “Major Changes of Von Willebrand Factor Multimer Distribution in Cirrhotic Patients with Stable Disease or Acute Decompensation.” Thrombosis and Haemostasis 118 (8): 1397–1408.
Vancouver
1.
Palyu E, Harsfalvi J, Tornai T, Papp M, Udvardy M, Szekeres-Csiki K, et al. Major changes of von Willebrand factor multimer distribution in cirrhotic patients with stable disease or acute decompensation. THROMBOSIS AND HAEMOSTASIS. 2018;118(8):1397–408.
IEEE
[1]
E. Palyu et al., “Major changes of von Willebrand factor multimer distribution in cirrhotic patients with stable disease or acute decompensation,” THROMBOSIS AND HAEMOSTASIS, vol. 118, no. 8, pp. 1397–1408, 2018.
@article{8571512,
  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.},
  author       = {Palyu, Eszter and Harsfalvi, Jolan and Tornai, Tamas and Papp, Maria and Udvardy, Miklos and Szekeres-Csiki, Katalin and Pataki, Lajos and Vanhoorelbeke, Karen and Feys, Hendrik and Deckmyn, Hans and Tornai, Istvan},
  issn         = {0340-6245},
  journal      = {THROMBOSIS AND HAEMOSTASIS},
  keywords     = {cirrhosis,acute decompensation,systemic inflammation,von Willebrand factor multimers,thrombotic micro-angiopathy,THROMBOTIC THROMBOCYTOPENIC PURPURA,ORTHOTOPIC LIVER-TRANSPLANTATION,FACTOR-CLEAVING PROTEASE,HEPATIC STELLATE CELLS,PLATELET ACTIVATION,HEPATOCELLULAR-CARCINOMA,BACTERIAL-INFECTIONS,VONWILLEBRAND-FACTOR,ADAMTS13,HEMOSTASIS},
  language     = {eng},
  number       = {8},
  pages        = {1397--1408},
  title        = {Major changes of von Willebrand factor multimer distribution in cirrhotic patients with stable disease or acute decompensation},
  url          = {http://dx.doi.org/10.1055/s-0038-1661393},
  volume       = {118},
  year         = {2018},
}

Altmetric
View in Altmetric
Web of Science
Times cited: