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

Elie Azoulay¹, Philippe R Bauer², Eric Mariotte³, Lene Russell⁴, Paul Knoebl⁵, Ignacio Martin-Loeches⁶, Frédéric Pène⁷, Kathryn Puxty⁸, Pedro Povoa^{9

10

11}, Andreas Barratt-Due¹², Jose Garnacho-Montero¹³, Julia Wendon¹⁴, Laveena Munshi¹⁵, Dominique Benoit¹⁶, Michael von Bergwelt-Baildon^{17

18}, Marco Maggiorini¹⁹, Paul Coppo²⁰, Spero Cataland²¹, Agnès Veyradier²², Andry Van de Louw²³; Nine-i Investigators

Affiliations

¹ Centre National Maladie rare des Microangiopathies Thrombotiques, Médecine Intensive et Réanimation, APHP, Saint-Louis Hospital and Paris University, Paris, France. elie.azoulay@aphp.fr.
² Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA.
³ Centre National Maladie rare des Microangiopathies Thrombotiques, Médecine Intensive et Réanimation, APHP, Saint-Louis Hospital and Paris University, Paris, France.
⁴ Department of Intensive Care, Copenhagen University Hospital, RigshospitaletCopenhagen Academy for Medical Simulation and Education, University of Copenhagen, and the Capital Region of Denmark, Copenhagen, Denmark.
⁵ Division of Hematology and Hemostasis Department of Medicine 1, Medical University of Vienna, Vienna, Austria.
⁶ Department of Intensive Care Medicine, Multidisciplinary Intensive Care Research Organization (MICRO), St. James's Hospital, St James Street, Dublin 8, Ireland.
⁷ Centre National Maladie Rare des Microangiopathies Thrombotiques, Médecine Intensive et Réanimation, Cochin Hospital and Paris University, Paris, France.
⁸ Department of Intensive Care, Glasgow Royal Infirmary, Glasgow, UK.
⁹ Polyvalent Intensive Care Unit, Hospital de São Francisco Xavier, Lisbon, Portugal.
¹⁰ NOVA Medical School, New University of Lisbon, Lisbon, Portugal.
¹¹ Center for Clinical Epidemiology and Research, Unit of Clinical Epidemiology, Odense University Hospital Odense, Odense, Denmark.
¹² Department of Emergencies and Critical Care, Oslo University Hospital, Oslo, Norway.
¹³ Intensive Care Clinical Unit, Hospital Universitario Virgen Macarena, Seville, Spain.
¹⁴ King's College Hospital, London, UK.
¹⁵ Interdepartmental Division of Critical Care Medicine, University of Toronto, Toronto, ON, Canada.
¹⁶ Ghent University Hospital, Ghent, Belgium.
¹⁷ Intensive Care in Hematologic and Oncologic Patients, Munich, Germany.
¹⁸ Medizinische Klinik und Poliklinik III, Klinikum der Universität München, Munich, Germany.
¹⁹ Medical Intensive Care Unit, University Hospital of Zurich, Zurich, Switzerland.
²⁰ Centre National Maladie Rare des Microangiopathies Thrombotiques, Service d'Hématologie, Saint-Antoine Hospital and Paris University, Paris, France.
²¹ Department of Medicine, Ohio State University, Columbus, OH, USA.
²² Centre National Maladies Rares des Microangiopathies Thrombotiques, Service d'Hématologie, Lariboisière Hospital and Paris University, Paris, France.
²³ Division of Pulmonary and Critical Care, Penn State University College of Medicine, Hershey, PA, USA.

PMID: 31588978
DOI: 10.1007/s00134-019-05736-5

Free article

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

Elie Azoulay et al. Intensive Care Med. 2019 Nov.

Free article

. 2019 Nov;45(11):1518-1539.

doi: 10.1007/s00134-019-05736-5. Epub 2019 Oct 7.

Authors

Affiliations

¹ Centre National Maladie rare des Microangiopathies Thrombotiques, Médecine Intensive et Réanimation, APHP, Saint-Louis Hospital and Paris University, Paris, France. elie.azoulay@aphp.fr.
² Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA.
³ Centre National Maladie rare des Microangiopathies Thrombotiques, Médecine Intensive et Réanimation, APHP, Saint-Louis Hospital and Paris University, Paris, France.
⁴ Department of Intensive Care, Copenhagen University Hospital, RigshospitaletCopenhagen Academy for Medical Simulation and Education, University of Copenhagen, and the Capital Region of Denmark, Copenhagen, Denmark.
⁵ Division of Hematology and Hemostasis Department of Medicine 1, Medical University of Vienna, Vienna, Austria.
⁶ Department of Intensive Care Medicine, Multidisciplinary Intensive Care Research Organization (MICRO), St. James's Hospital, St James Street, Dublin 8, Ireland.
⁷ Centre National Maladie Rare des Microangiopathies Thrombotiques, Médecine Intensive et Réanimation, Cochin Hospital and Paris University, Paris, France.
⁸ Department of Intensive Care, Glasgow Royal Infirmary, Glasgow, UK.
⁹ Polyvalent Intensive Care Unit, Hospital de São Francisco Xavier, Lisbon, Portugal.
¹⁰ NOVA Medical School, New University of Lisbon, Lisbon, Portugal.
¹¹ Center for Clinical Epidemiology and Research, Unit of Clinical Epidemiology, Odense University Hospital Odense, Odense, Denmark.
¹² Department of Emergencies and Critical Care, Oslo University Hospital, Oslo, Norway.
¹³ Intensive Care Clinical Unit, Hospital Universitario Virgen Macarena, Seville, Spain.
¹⁴ King's College Hospital, London, UK.
¹⁵ Interdepartmental Division of Critical Care Medicine, University of Toronto, Toronto, ON, Canada.
¹⁶ Ghent University Hospital, Ghent, Belgium.
¹⁷ Intensive Care in Hematologic and Oncologic Patients, Munich, Germany.
¹⁸ Medizinische Klinik und Poliklinik III, Klinikum der Universität München, Munich, Germany.
¹⁹ Medical Intensive Care Unit, University Hospital of Zurich, Zurich, Switzerland.
²⁰ Centre National Maladie Rare des Microangiopathies Thrombotiques, Service d'Hématologie, Saint-Antoine Hospital and Paris University, Paris, France.
²¹ Department of Medicine, Ohio State University, Columbus, OH, USA.
²² Centre National Maladies Rares des Microangiopathies Thrombotiques, Service d'Hématologie, Lariboisière Hospital and Paris University, Paris, France.
²³ Division of Pulmonary and Critical Care, Penn State University College of Medicine, Hershey, PA, USA.

PMID: 31588978
DOI: 10.1007/s00134-019-05736-5

Erratum in

Correction to: Expert statement on the ICU management of patients with thrombotic thrombocytopenic purpura.
Azoulay E, Bauer PR, Mariotte E, Russell L, Knoebl P, Martin-Loeches I, Pène F, Puxty K, Povoa P, Barratt-Due A, Garnacho-Montero J, Wendon J, Munshi L, Benoit D, von Bergwelt-Baildon M, Maggiorini M, Coppo P, Cataland S, Veyradier A, Van de Louw A; Nine-i Investigators. Azoulay E, et al. Intensive Care Med. 2020 Mar;46(3):570-571. doi: 10.1007/s00134-019-05904-7. Intensive Care Med. 2020. PMID: 31965263

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

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

Affiliations

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

Authors

Affiliations

Erratum in

Abstract

References

MeSH terms

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LinkOut - more resources

Full Text Sources

Research Materials