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Updated: Aug 10, 2026
Technique of Porcine Liver Procurement and Orthotopic Transplantation using an Active Porto-Caval Shunt
Published on: May 7, 2015
Plasma ADAMTS13 activity may predict early adverse events in living donor liver transplantation: observations in 3
Saiho Ko1, Eiji Okano, Hiromichi Kanehiro
1Department of Surgery, Nara Medical University, Kashihara, Nara, Japan. saihoko@naramed-u.ac.jp
Abstract:
A disintegrin-like and metalloproteinase with thrombospondin type-1 motifs 13 (ADAMTS13) is a metalloproteinase that specifically cleaves the multimeric von Willebrand factor (VWF). Deficiency of ADAMTS13 increases the unusually large VWF multimers (UL-VWFM), which leads to platelet clumping and/or thrombus formation, resulting in microcirculatory disturbance. We serially determined the activity of plasma ADAMTS13, together with VWF antigen (VWF:Ag) and UL-VWFM, in association with the development of early graft dysfunction in 3 liver transplant recipients and 4 patients with major hepatectomy as controls. In case 1, ADAMTS13 activity decreased markedly from 108% to less than 3% with concomitant thrombocytopenia on posttransplantation day 7, when acute rejection occurred. Simultaneously, UL-VWFM were detected. During the second episode of rejection, VWF:Ag increased to 368% with the appearance of UL-VWFM, while ADAMTS13 activity was as low as 18%, indicating an imbalance between a large amount of UL-VWFM and low activity of ADAMTS13. Administration of fresh frozen plasma (FFP) together with treatment for acute rejection resulted in an improvement of ADAMTS13 activity and disappearance of the UL-VWFM. In case 2, ADAMTS13 activity promptly decreased to 9% with thrombocytopenia on day 1, when ischemia-reperfusion injury occurred. Subsequently, the ADAMTS13 activity increased steadily without appearance of UL-VWFM, and the patient recovered uneventfully. ADAMTS13 activity decreased to 15% immediately after transplantation in case 3 as well. In contrast, ADAMTS13 activity never decreased below 20% in 4 patients with major hepatectomy as controls. In conclusion, these results indicate that the kinetics of ADAMTS13 and UL-VWFM could be good indicators of adverse events after liver transplantation. Our findings not only suggest a novel mechanism for thrombocytopenia, but also provide a useful tool for diagnosis of graft dysfunction in the early stage after transplantation.
Insights
Low ADAMTS13 activity and increased unusually large von Willebrand factor multimers (UL-VWFM) indicate early graft dysfunction after liver transplantation. Monitoring these factors aids in diagnosing complications and understanding thrombocytopenia mechanisms.
Area of Science:
- Biochemistry
- Hematology
- Transplantation Medicine
Background:
- ADAMTS13 (A disintegrin-like and metalloproteinase with thrombospondin type-1 motifs 13) cleaves von Willebrand factor (VWF).
- ADAMTS13 deficiency leads to unusually large VWF multimers (UL-VWFM), causing platelet aggregation and microcirculatory issues.
- Early graft dysfunction after liver transplantation is a critical clinical concern.
Observation:
- Serial measurements of ADAMTS13 activity, VWF antigen (VWF:Ag), and UL-VWFM were performed in liver transplant recipients and hepatectomy controls.
- Liver transplant recipients experiencing acute rejection or ischemia-reperfusion injury showed marked decreases in ADAMTS13 activity and the presence of UL-VWFM.
- One patient with severe rejection had critically low ADAMTS13 activity (3%) and UL-VWFM, which improved after fresh frozen plasma (FFP) administration and rejection treatment.
Findings:
- ADAMTS13 activity kinetics and UL-VWFM levels correlated with adverse events, including thrombocytopenia and graft dysfunction, in liver transplant recipients.
- A significant imbalance between high UL-VWFM levels and low ADAMTS13 activity was observed during rejection episodes.
- ADAMTS13 activity remained above 20% in major hepatectomy controls, suggesting transplantation-specific effects.
Implications:
- The interplay between ADAMTS13 and UL-VWFM serves as a potential biomarker for early detection of liver graft dysfunction.
- These findings suggest a novel mechanism contributing to thrombocytopenia post-liver transplantation.
- Monitoring ADAMTS13 and UL-VWFM offers a valuable diagnostic tool for managing early complications in liver transplant patients.
