Scholarlink

Background <p>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.</p> Methods <p>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.</p> Results <p>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 (<i>p</i> = 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% (<i>n</i> = 8/42) in the SOC group, while it was 36.4% (<i>n</i> = 8/22) in patients receiving adjunctive PEX (Gehan-Breslow-Wilcoxon-<i>p</i> = 0.041, Log-Rank-<i>p</i> = 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, <i>p</i> = 0.004). After propensity-score-matching, LTX-free survival was 28% in the SOC- and 52% in the PEX group (Gehan-Breslow-<i>p</i> = 0.036; Log-Rank-<i>p</i> = 0.035).</p> Conclusions <p>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.</p>

错误：搜索内容不能为空，请输入英文关键词
错误：关键词超出字数限制，请精简
高级检索

Therapeutic plasma exchange in amatoxin associated acute liver failure–results from the multi-center Amanita-PEX study

  • 期刊论文

  • 出版模式：

    开放获取

  • 发表日期：

    2025年10月30日

查看全文
  • 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,
  • Benjamin Seeliger,
  • Kai-Hendrik Peiffer,
  • Petra Stöckert,
  • Sascha David,
  • Rea Andermatt,
  • Markus Busch,
  • Olaf Wiesner,
  • Benjamin Maasoumy,
  • Rene Abu-Isneineh,
  • 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

摘要

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.