Dissecting the pathophysiology of immune thrombotic thrombocytopenic purpura

Dissecting the pathophysiology of immune thrombotic thrombocytopenic purpura: interplay between genes and environmental triggers by Johana Hrdinová, Silvia D’Angelo, Nuno A. G. Graça, Bogac Ercig, Karen Vanhoorelbeke, Agnès Veyradier, Jan Voorberg, and Paul Coppo haematol Volume 103(7): 1099 -1109 July 3, 2018 © 2018 by Ferrata Storti Foundation

The genes of the human MHC locus involved in immune-mediated thrombotic thrombocytopenic purpura (i. TTP) susceptibility. Johana Hrdinová et al. Haematologica 2018; 103: 1099 -1109 © 2018 by Ferrata Storti Foundation

The possible role of MHC class II stability in the onset of immune-mediated thrombotic thrombocytopenic purpura (i. TTP). Johana Hrdinová et al. Haematologica 2018; 103: 1099 -1109 © 2018 by Ferrata Storti Foundation

The onset of immune-mediated thrombotic thrombocytopenic purpura (i. TTP). Johana Hrdinová et al. Haematologica 2018; 103: 1099 -1109 © 2018 by Ferrata Storti Foundation

Conformational changes of ADAMTS 13. [Mp: metalloprotease (red); Dis: disintegrin-like domain (green); Cys-rich: cysteine-rich domain (orange); TSRs: thrombospondin-type 1 repeats (light and dark gray); CUB 1 domain (cyan); CUB 2 domain (magenta)]. Johana Hrdinová et al. Haematologica 2018; 103: 1099 -1109 © 2018 by Ferrata Storti Foundation

Presentation of ADAMTS 13 peptides on MHC class II. (A) Three-dimensional model structure of HLA-DRB 1*11 (graphic representation in gray) with FINVAPHAR peptide (stick representation in magenta with cyan surface) from ADAMTS 13 CUB 2 domain. Johana Hrdinová et al. Haematologica 2018; 103: 1099 -1109 © 2018 by Ferrata Storti Foundation