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|Title:||Link between von Willebrand factor multimers, relapses and coronary microcirculation in patients with thrombotic thrombocytopenic purpura in remission||Authors:||Di Pasquale, Irene
Lombardi, Anna Maria
|Keywords:||ADAMTS13;Thrombotic thrombocytopenic purpura (TTP);von Willebrand factor||Keywords Plus:||FACTOR-CLEAVING PROTEASE;FLOW RESERVE;MICROVASCULAR DYSFUNCTION;VONWILLEBRAND-FACTOR;EARLY ATHEROSCLEROSIS;CARDIAC INVOLVEMENT;ADAMTS13;DISEASE;DIAGNOSIS;MICROANGIOPATHY||Mesh headings:||Coronary Vessels;Microcirculation;Protein Multimerization;Purpura, Thrombotic Thrombocytopenic;von Willebrand Factor||Secondary Mesh headings:||ADAMTS13 Protein;Adult;Female;Hemostasis;Humans;Male;Middle Aged;Prospective Studies;Recurrence;Remission Induction||Issue Date:||2019||Publisher:||PERGAMON-ELSEVIER SCIENCE LTD||Journal:||Thrombosis research||Abstract:||
ADAMTS13 deficiency results in unusually large von Willebrand factor (ULVWF) multimers in the circulation and a higher risk of microthrombi due to high shear stress. In patients treated for acquired thrombotic thrombocytopenic purpura (TTP), a persistently severe ADAMTS13 deficiency (<10%) in remission is associated with more relapses. A reduced plasma ADAMTS13 activity and increased VWF levels are associated with a higher risk of myocardial infarction. Assessing coronary flow reserve (CFR) enables a better cardiovascular risk stratification: a lower CFR correlates inversely with cardiovascular risk. The aim of the study was to establish whether patients with TTP in remission have an impaired coronary microcirculation, in terms of a lower CFR, and whether there is any correlation between ADAMTS13 activity, the presence of ULVWF multimers, and the occurrence of relapses.
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