Therapeutic plasma exchange in amatoxin associated acute liver failure-results from the multi-center Amanita-PEX study
Background: Amatoxin-related acute liver failure (AT-ALF) carries high mortality without liver transplantation (LTX). While therapeutic plasma exchange (PEX) might improve LTX-free survival in other ALF cases, its role in AT-ALF is unclear. Clinical practice varies, and, given the rarity of this ALF...
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| Main Authors: | , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , |
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| Format: | Article (Journal) |
| Language: | English |
| Published: |
30 October 2025
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| In: |
Critical care
Year: 2025, Volume: 29, Pages: 1-16 |
| ISSN: | 1466-609X |
| DOI: | 10.1186/s13054-025-05560-y |
| Online Access: | Verlag, kostenfrei, Volltext: https://doi.org/10.1186/s13054-025-05560-y
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| Author Notes: | Klaus Stahl, Bahar Nalbant, Thorben Pape, Isaure Breteau, Valentin Coirier, Filipe S. Cardoso, Jubi de Haan, Maciej K. Janik, Jan-Christian Wasmuth, João Madaleno, Uta Merle, Josephine Frohme, Phil-Robin Tepasse, Martina Müller, Karsten Große, Alexandra Linke, Nikola Mareljic, Fin Stolze Larsen, Gérladine Dahlqvist, Mirjam Kolev, Marie Schulze, Katharina Willuweit, Petra Janke-Maier, Felix Dondorf, Óscar M. Fierro-Angulo, Anja Geerts, David Toapanta, Camille Dejean, Mohamed Alharthi, Enric Reverter, Heiko Schenk, Sarah Raevens, Ricardo Ulises Macías-Rodríguez, Falk Rauchfuß, Christoph P. Berg, Hartmut Schmidt, Andreas Geier, Nasser Semmo, Nicolas Lanthier, Peter N. Bjerring, Christian M. Lange, Martina Sterneck, Tony Bruns, Stephan Schmid, Dominik van de Loo, Münevver Demir, Tobias Boettler, Catarina Borges, Jacob Nattermann, Karolina Wronka, Caroline M. den Hoed, Hugo P. Marques, Florent Artru, Eric Levesque, Heiner Wedemeyer, Alejandro Campos-Murguia, Richard Taubert and for the Amanita-PEX-study group |
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| 245 | 1 | 0 | |a Therapeutic plasma exchange in amatoxin associated acute liver failure-results from the multi-center Amanita-PEX study |c Klaus Stahl, Bahar Nalbant, Thorben Pape, Isaure Breteau, Valentin Coirier, Filipe S. Cardoso, Jubi de Haan, Maciej K. Janik, Jan-Christian Wasmuth, João Madaleno, Uta Merle, Josephine Frohme, Phil-Robin Tepasse, Martina Müller, Karsten Große, Alexandra Linke, Nikola Mareljic, Fin Stolze Larsen, Gérladine Dahlqvist, Mirjam Kolev, Marie Schulze, Katharina Willuweit, Petra Janke-Maier, Felix Dondorf, Óscar M. Fierro-Angulo, Anja Geerts, David Toapanta, Camille Dejean, Mohamed Alharthi, Enric Reverter, Heiko Schenk, Sarah Raevens, Ricardo Ulises Macías-Rodríguez, Falk Rauchfuß, Christoph P. Berg, Hartmut Schmidt, Andreas Geier, Nasser Semmo, Nicolas Lanthier, Peter N. Bjerring, Christian M. Lange, Martina Sterneck, Tony Bruns, Stephan Schmid, Dominik van de Loo, Münevver Demir, Tobias Boettler, Catarina Borges, Jacob Nattermann, Karolina Wronka, Caroline M. den Hoed, Hugo P. Marques, Florent Artru, Eric Levesque, Heiner Wedemeyer, Alejandro Campos-Murguia, Richard Taubert and for the Amanita-PEX-study group |
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| 520 | |a Background: Amatoxin-related acute liver failure (AT-ALF) carries high mortality without liver transplantation (LTX). While therapeutic plasma exchange (PEX) might improve LTX-free survival in other ALF cases, its role in AT-ALF is unclear. Clinical practice varies, and, given the rarity of this ALF entity, the feasibility of conducting a randomized controlled trial to investigate PEX in AT-ALF is more or less impossible. Methods: The Amanita-PEX study is a multi-center, international, retrospective study analyzing patients with AT-ALF from 2013 to 2024. The primary outcome was 28-day LTX-free survival (composite endpoint: death or LTX) after ALF diagnosis. Results: The study included 111 patients from 25 centers: 82 received standard-of-care (SOC), and 29 received at least one PEX-session. PEX and SOC-groups were comparable at baseline, but 76% of PEX- vs. 58% of SOC-patients developed hepatic-encephalopathy (HE) grade ≥ 2 (p = 0.021). While the primary outcome of 28-day LTX-free survival in all patients was not different between the SOC and PEX-groups, in the subgroup of patients with maximal HE grade ≥ 2, LTX-free survival was 19.1% (n = 8/42) in the SOC group, while it was 36.4% (n = 8/22) in patients receiving adjunctive PEX (Gehan-Breslow-Wilcoxon-p = 0.041, Log-Rank-p = 0.060). PEX was independently associated with reduced risk of the combined endpoint death or liver transplantation within 28 days from inclusion in patients with HE grade ≥ 2 (HR 0.37, 95%-CI 0.19–0.73, p = 0.004). After propensity-score-matching, LTX-free survival was 28% in the SOC- and 52% in the PEX group (Gehan-Breslow-p = 0.036; Log-Rank-p = 0.035). Conclusions: In this real-world study, adjunctive use of PEX was associated with increased LTX-free-survival in patients with AT-ALF and HE grade ≥ 2. | ||
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