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Erschienen in: Intensive Care Medicine 6/2018

11.05.2018 | Letter

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

verfasst von: 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

Erschienen in: Intensive Care Medicine | Ausgabe 6/2018

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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]. …
Literatur
1.
Zurück zum Zitat 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 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
3.
Zurück zum Zitat 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 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
Metadaten
Titel
The clinical features of cardiac involvement in patients with severe thrombotic thrombocytopenic purpura
verfasst von
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
Publikationsdatum
11.05.2018
Verlag
Springer Berlin Heidelberg
Erschienen in
Intensive Care Medicine / Ausgabe 6/2018
Print ISSN: 0342-4642
Elektronische ISSN: 1432-1238
DOI
https://doi.org/10.1007/s00134-018-5216-7

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