Contribution of ADAMTS13-independent VWF regulation in sickle cell disease.

ABSTRACT:

Background

Von Willebrand factor (VWF) is elevated in sickle cell disease (SCD) and contributes to vaso-occlusion through its thrombogenic properties. VWF is regulated by ADAMTS13, a plasma protease that cleaves VWF into less bioactive multimers. Independent investigations have shown VWF to be elevated in SCD, whereas measurements of ADAMTS13 have been variable.

Objectives

We assessed ADAMTS13 activity using multiple activity assays and measured levels of alternative VWF-cleaving proteases in SCD.

Methods/ patients

Plasma samples were collected from adult patients with SCD (n = 20) at a single institution when presenting for routine red cell exchange transfusion therapy. ADAMTS13 activity was measured by FRETS-VWF73, Technozym ADAMTS-13 Activity ELISA kit and a full-l

SUBMITTER: Hunt RC 

PROVIDER: S-EPMC10460119 | biostudies-literature | 2022 Sep

REPOSITORIES: biostudies-literature