by J. Evan Sadler Blood Volume 112(1):11-18 July 1, 2008

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Presentation transcript:

Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura by J. Evan Sadler Blood Volume 112(1):11-18 July 1, 2008 ©2008 by American Society of Hematology

Evan Sadler. Evan Sadler J. Evan Sadler Blood 2008;112:11-18 ©2008 by American Society of Hematology

Structure of ADAMTS13. Structure of ADAMTS13. The primary translation product consists of 1427 amino acid residues. Motifs include a signal peptide (S), propeptide (P), metalloprotease (M), disintegrin (D), Cys-rich, spacer, CUB, and thrombospondin (TSP1) domains (1-8). J. Evan Sadler Blood 2008;112:11-18 ©2008 by American Society of Hematology

Pathogenesis of idiopathic TTP caused by ADAMTS13 deficiency. Pathogenesis of idiopathic TTP caused by ADAMTS13 deficiency. Multimeric VWF adheres to endothelial cells or to connective tissue exposed in the vessel wall. Platelets adhere to VWF through platelet membrane GPIb. In flowing blood, VWF in the platelet-rich thrombus is stretched and cleaved by ADAMTS13, limiting thrombus growth. If ADAMTS13 is absent, VWF-dependent platelet accumulation continues, eventually causing microvascular thrombosis and TTP. J. Evan Sadler Blood 2008;112:11-18 ©2008 by American Society of Hematology