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

2019; Springer Science+Business Media; Volume: 45; Issue: 11 Linguagem: Inglês

10.1007/s00134-019-05736-5

1432-1238

Élie Azoulay, Philippe R. Bauer, Éric Mariotte, Lene Russell, Paul Knoebl, Ignacio Martín‐Loeches, Frédéric Pène, Kathryn Puxty, Pedro Póvoa, Andreas Barratt‐Due, José Garnacho‐Montero, Julia Wendon, Laveena Munshi, Dominique Benoît, Michael von Bergwelt‐Baildon, Marco Maggiorini, Paul Coppo, Spero R. Cataland, Agnès Veyradier, Andry Van de Louw,

Platelet Disorders and Treatments

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.