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

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 variabilit...

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Bibliographic Details
Published in:Intensive care medicine 2019-11, Vol.45 (11), p.1518-1539
Main Authors: Azoulay, Elie, Bauer, Philippe R., Mariotte, Eric, Russell, Lene, Knoebl, Paul, Martin-Loeches, Ignacio, Pène, Frédéric, Puxty, Kathryn, Povoa, Pedro, Barratt-Due, Andreas, Garnacho-Montero, Jose, Wendon, Julia, Munshi, Laveena, Benoit, Dominique, von Bergwelt-Baildon, Michael, Maggiorini, Marco, Coppo, Paul, Cataland, Spero, Veyradier, Agnès, Van de Louw, Andry
Format: Article
Language:English
Subjects:
Anesthesiology
Apheresis
Critical Care Medicine
Emergency Medicine
Immunotherapy
Intensive
Intensive care
Management
Medicine
Medicine & Public Health
Monoclonal antibodies
Pain Medicine
Patients
Pediatrics
Pneumology/Respiratory System
Purpura
Rituximab
Seven-Day Profile Publication
Thrombocytopenic purpura
Thrombotic thrombocytopenic purpura
Trustworthiness
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Summary: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.
ISSN:0342-4642
1432-1238
DOI:10.1007/s00134-019-05736-5