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01-06-2018 | Letter

The clinical features of cardiac involvement in patients with severe thrombotic thrombocytopenic purpura

Authors: Aude-Marie Fourmont, Lara Zafrani, Eric Mariotte, Lionel Galicier, Bérangère Joly, Sybille Merceron, Rémi Bertinchamp, Virginie Lemiale, Audrey de Jong, Sandrine Valade, Michael Darmon, Agnès Veyradier, Elie Azoulay

Published in: Intensive Care Medicine | Issue 6/2018

Excerpt

Thrombotic thrombocytopenic purpura (TTP) is a rare life-threatening microangiopathic syndrome [1]. Mortality can reach 20% [1]. A severe deficit in ADAMTS13 (a disintegrin and metalloprotease with thrombospodin type I repeat 13, which cleaves the von Willebrand factor, vWF) results in the accumulation of unfolded high molecular weight multimers that activate the endothelium and aggregate with platelets in the microvessels. Platelet–vWF thrombi cause ischemic organ failure [2]. TTP-related multiorgan dysfunction primarily affects the heart and the brain [1]. …

Mariotte E, Azoulay E, Galicier L et al (2016) Epidemiology and pathophysiology of adulthood-onset thrombotic microangiopathy with severe ADAMTS13 deficiency (thrombotic thrombocytopenic purpura): a cross-sectional analysis of the French national registry for thrombotic microangiopathy. Lancet Haematol 3:e237–245. https://doi.org/10.1016/S2352-3026(16)30018-7 CrossRefPubMed

Mannucci PM (2015) Understanding organ dysfunction in thrombotic thrombocytopenic purpura. Intensive Care Med 41:715–718. https://doi.org/10.1007/s00134-014-3630-z CrossRefPubMed

Benhamou Y, Boelle P-Y, Baudin B et al (2015) Cardiac troponin-I on diagnosis predicts early death and refractoriness in acquired thrombotic thrombocytopenic purpura. Experience of the French Thrombotic Microangiopathies Reference Center. J Thromb Haemost 13:293–302. https://doi.org/10.1111/jth.12790 CrossRefPubMed

Sane DC, Streer NP, Owen J (2009) Myocardial necrosis in patients with thrombotic thrombocytopenic purpura: pathophysiology and rationale for specific therapy. Eur J Haematol 82:83–92. https://doi.org/10.1111/j.1600-0609.2008.01172.x CrossRefPubMed

Mariotte E, Blet A, Galicier L et al (2013) Unresponsive thrombotic thrombocytopenic purpura in critically ill adults. Intensive Care Med 39:1272–1281. https://doi.org/10.1007/s00134-013-2873-4 CrossRefPubMed

Title: The clinical features of cardiac involvement in patients with severe thrombotic thrombocytopenic purpura
Authors: Aude-Marie Fourmont
Lara Zafrani
Eric Mariotte
Lionel Galicier
Bérangère Joly
Sybille Merceron
Rémi Bertinchamp
Virginie Lemiale
Audrey de Jong
Sandrine Valade
Michael Darmon
Agnès Veyradier
Elie Azoulay
Publication date: 01-06-2018
Publisher: Springer Berlin Heidelberg
Published in: Intensive Care Medicine / Issue 6/2018
Print ISSN: 0342-4642
Electronic ISSN: 1432-1238
DOI: https://doi.org/10.1007/s00134-018-5216-7

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The clinical features of cardiac involvement in patients with severe thrombotic thrombocytopenic purpura

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