Expert statement on the ICU management of patients with thrombotic thrombocytopenic purpura

Elie Azoulay, Philippe R. Bauer, Eric Mariotte, Lene Russell, Paul Knoebl, Ignacio Martin-Loeches, Frédéric Pène, Kathryn Puxty, Pedro Povoa, Andreas Barratt-Due, Jose Garnacho-Montero, Julia Wendon, Laveena Munshi, Dominique Benoit, Michael von Bergwelt-Baildon, Marco Maggiorini, Paul Coppo, Spero Cataland, Agnès Veyradier, Andry Van de Louw

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19 Citations (Scopus)


Thrombotic thrombocytopenic purpura (TTP) is fatal in 90% of patients if left untreated and must be diagnosed early to optimize patient outcomes. However, the very low incidence of TTP is an obstacle to the development of evidence-based clinical practice recommendations, and the very wide variability in survival rates across centers may be partly ascribable to differences in management strategies due to insufficient guidance. We therefore developed an expert statement to provide trustworthy guidance about the management of critically ill patients with TTP. As strong evidence was difficult to find in the literature, consensus building among experts could not be reported for most of the items. This expert statement is timely given the recent advances in the treatment of TTP, such as the use of rituximab and of the recently licensed drug caplacizumab, whose benefits will be maximized if the other components of the management strategy follow a standardized pattern. Finally, unanswered questions are identified as topics of future research on TTP.

Original languageEnglish
Pages (from-to)1518-1539
Number of pages22
JournalIntensive Care Medicine
Issue number11
Publication statusPublished - 1 Nov 2019


  • Acute kidney injury
  • Auto-immune disease
  • Cardiac failure
  • Hemolysis
  • Plasma exchange
  • Thrombocytopenia


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