Intensive Care Med (2017) 43:1398–1400 DOI 10.1007/s00134-016-4662-3 UNDERSTANDING THE DISEASE Understanding therapeutic targets in thrombotic thrombocytopenic purpura 1 2 1* Bérangère S. Joly , Karen Vanhoorelbeke and Agnès Veyradier © 2017 Springer-Verlag Berlin Heidelberg and ESICM Thrombotic thrombocytopenic purpura (TTP) is a rare and multiple organ failure [5, 6]. The mechanism of thrombotic microangiopathy specifically related to a severe ADAMTS13 deficiency is acquired in the large severe functional deficiency of the von Willebrand factor majority of cases and is related to the presence of anti- (VWF) cleaving protease, ADAMTS13 (a disintegrin and ADAMTS13 auto-antibodies (auto-Abs). In some rare metalloprotease with thrombospondin type 1 repeats, cases, ADAMTS13 deficiency is inherited, and is linked member 13) [1, 2]. VWF is a multimeric plasma glyco- to the presence of bi-allelic recessive mutations in the protein of which the monomers, containing multiple ADAMTS13 gene [1–3, 5–8]. Whatever the acquired or functional domains, are connected by disulfide bonds [ 3]. hereditary etiology of TTP, its time course is character- In cases of vascular injury, VWF contributes to the initial ized by a relapsing tendency. Acute TTP remains a severe recruitment of platelets to the injured vessel wall and to and life-threatening disease, but treatments, most
Intensive Care Medicine – Springer Journals
Published: Jan 23, 2017
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