Published in:
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
Login to get access
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]. …