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The Effect of Liver Transplantation on ADAMTS13 Activity and Antigen Levels

1University Medical Center of the Johannes-Gutenberg University Mainz, Institute of Clinical Chemistry and Laboratory Medicine, Mainz, Germany, 2University Medical Center of the Johannes Gutenberg University, Institute of Clinical Chemistry and Laboratory Medicine, Mainz, Germany, 3University Medical Center of the Johannes Gutenberg University, Department of Internal Medicine, Mainz, Germany, 4University Medical Center of the Johannes-Gutenberg University Mainz, Center for Thrombosis and Hemostasis, Mainz, Germany, 5Inselspital Bern University Hospital University of Bern, Department of Hematology and Central Hematology Laboratory, Bern, Switzerland, 6University Collage London, Haemostasis Research Unit, London, United Kingdom, 7University Medical Center of the Johannes Gutenberg University, Department of General and Abdominal Surgery, Mainz, Germany

Abstract Number: PB1880

Meeting: ISTH 2020 Congress

Theme: Thrombotic Microangiopathies » ADAMTS13 and TTP

Background: The liver plays a central role in the promotion and regulation of hemostasis. The VWF-cleaving protease – ADAMTS13 – is mainly produced by hepatic stellate cells. Deficiency of ADAMTS13 activity results in accumulation of unusually large VWF multimers, leading to platelet adhesion and aggregation in the microvasculature and organ ischemia causing thrombotic microangiopathy (TMA).

Aims: The aim of this study was to characterize the effect of liver transplantation (LTX) on ADAMTS13 activity and ADAMTS13 antigen.

Methods: In this prospective observational study, 25 patients (9 females, 16 males) were included who were consecutively transplanted between April 2017 and December 2017 in our center. A laboratory “TMA panel” including ADAMTS13 activity, ADAMTS13 antigen levels, hemoglobin, haptoglobin, LDH and fragmentocytes in addition to routine laboratory liver transplant testing was performed before, 2-4 days, 7-9 days and during the first outpatient visit (median 14 weeks after LTX) after liver transplantation.

Results: After LTX, in 16 patients a decreased ADAMTS13 antigen level as well as a decreased ADAMTS13 activity (median 43.88% [IQR 37.03-46.03, min 22.67, max 49.08]) was detectable.
Three patients showed signs of a TMA (platelets < 100/nl, hemoglobin (females < 12g/dl/ males < 14g/dl, haptoglobin < 0.35g/l, increased LDH levels >254U/l and fragmentocytes >5‰). One patient died as a result of septic shock with subsequent multi-organ failure.
All surviving patients had an ADAMST13 activity and antigen values of median 81.75% [IQR 58.60-97.80, min 52.2] and median 0.768ng/ml [IQR 0.499-0.884] at the first outpatient visit.

Conclusions: In summary, the occurrence of TMA during liver transplantation is rare, whereas a moderate decrease of ADAMTS13 activity and ADAMTS13 antigen occurs in more than half of liver transplant recipients. Nevertheless, in patients after liver transplantation with decreased platelets and hemolytic anemia, TMA should be considered and ADAMTS13 activity should be analysed to rule out TMA.

To cite this abstract in AMA style:

Falter T, Häuser F, Hofsaess F, Greulich L, Sprinzl M, Lackner K, Scharrer I, Lämmle B, Mittler J. The Effect of Liver Transplantation on ADAMTS13 Activity and Antigen Levels [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/the-effect-of-liver-transplantation-on-adamts13-activity-and-antigen-levels/. Accessed September 24, 2023.

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