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Intensive Care Medicine

, Volume 45, Issue 11, pp 1518–1539 | Cite as

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

  • Elie AzoulayEmail author
  • 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
  • On behalf of the Nine-i Investigators
Seven-Day Profile Publication

Abstract

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.

Keywords

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

Notes

Author contributions

All experts contributed significantly to developing the statement and to writing and revising the manuscript. All experts have approved the final submitted version.

Compliance with ethical standards

Conflicts of interest

This statement is the result of independent work carried out by members of the Nine-i network of investigators. None of the authors received any honoraria or fees for this work. The Nine-i network has received research grants and sponsorships from Gilead, Fisher&Payckle, Jazz Pharma, Ablynx, Baxter, Alexion, and Astellas. Financial ties unrelated to this work are as follows. Over the last 3 years, EA received honoraria or travel expense reimbursements from Alexion, Baxter, MSD, Ablynx, Pfizer, and Gilead. IML has received honoraria or travel expense reimbursements from Biomerieux, MSD, and Gilead. AV sits on the advisory boards of Ablynx-Sanofi-Genzyme, Roche-Chugai, and Shire-Takeda. PC sits on the advisory boards of Alexion, Sanofi-Genzyme, Roche-Chugai, Octapharma, and Shire-Takeda. PP received honoraria or travel expense reimbursements from Pfizer and Orion. DB has received grants from Gilead, Astellas, Fisher-Paykel, Baxter, Alexion, and Fresenius Kabi. SC sits on the advisory boards of Alexion, Sanofi-Genzyme, and Shire-Takeda. FP and JGM have received fees from Alexion. None of the other authors has any potential conflicts of interest to declare.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  • Elie Azoulay
    • 1
    Email author
  • Philippe R. Bauer
    • 2
  • Eric Mariotte
    • 1
  • Lene Russell
    • 3
  • Paul Knoebl
    • 4
  • Ignacio Martin-Loeches
    • 5
  • Frédéric Pène
    • 6
  • Kathryn Puxty
    • 7
  • Pedro Povoa
    • 8
    • 9
    • 10
  • Andreas Barratt-Due
    • 11
  • Jose Garnacho-Montero
    • 12
  • Julia Wendon
    • 13
  • Laveena Munshi
    • 14
  • Dominique Benoit
    • 15
  • Michael von Bergwelt-Baildon
    • 16
    • 17
  • Marco Maggiorini
    • 18
  • Paul Coppo
    • 20
  • Spero Cataland
    • 21
  • Agnès Veyradier
    • 22
  • Andry Van de Louw
    • 19
  • On behalf of the Nine-i Investigators
  1. 1.Centre National Maladie rare des Microangiopathies Thrombotiques, Médecine Intensive et RéanimationAPHP, Saint-Louis Hospital and Paris UniversityParisFrance
  2. 2.Division of Pulmonary and Critical Care MedicineMayo ClinicRochesterUSA
  3. 3.Department of Intensive CareCopenhagen University Hospital, RigshospitaletCopenhagen Academy for Medical Simulation and Education, University of Copenhagen, and the Capital Region of DenmarkCopenhagenDenmark
  4. 4.Division of Hematology and Hemostasis Department of Medicine 1Medical University of ViennaViennaAustria
  5. 5.Department of Intensive Care MedicineMultidisciplinary Intensive Care Research Organization (MICRO), St. James’s HospitalDublin 8Ireland
  6. 6.Centre National Maladie Rare des Microangiopathies Thrombotiques, Médecine Intensive et RéanimationCochin Hospital and Paris UniversityParisFrance
  7. 7.Department of Intensive CareGlasgow Royal InfirmaryGlasgowUK
  8. 8.Polyvalent Intensive Care UnitHospital de São Francisco XavierLisbonPortugal
  9. 9.NOVA Medical SchoolNew University of LisbonLisbonPortugal
  10. 10.Center for Clinical Epidemiology and Research, Unit of Clinical EpidemiologyOdense University Hospital OdenseOdenseDenmark
  11. 11.Department of Emergencies and Critical CareOslo University HospitalOsloNorway
  12. 12.Intensive Care Clinical UnitHospital Universitario Virgen MacarenaSevilleSpain
  13. 13.King’s College HospitalLondonUK
  14. 14.Interdepartmental Division of Critical Care MedicineUniversity of TorontoTorontoCanada
  15. 15.Ghent University HospitalGhentBelgium
  16. 16.Intensive Care in Hematologic and Oncologic PatientsMunichGermany
  17. 17.Medizinische Klinik und Poliklinik IIIKlinikum der Universität MünchenMunichGermany
  18. 18.Medical Intensive Care UnitUniversity Hospital of ZurichZurichSwitzerland
  19. 19.Division of Pulmonary and Critical CarePenn State University College of MedicineHersheyUSA
  20. 20.Centre National Maladie Rare des Microangiopathies Thrombotiques, Service d’HématologieSaint-Antoine Hospital and Paris UniversityParisFrance
  21. 21.Department of MedicineOhio State UniversityColumbusUSA
  22. 22.Centre National Maladies Rares des Microangiopathies Thrombotiques, Service d’HématologieLariboisière Hospital and Paris UniversityParisFrance

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