학술논문
ADAMTS13 and von Willebrand factor assessment in steady state and acute vaso‐occlusive crisis of sickle cell disease
Document Type
article
Author
Source
Research and Practice in Thrombosis and Haemostasis, Vol 5, Iss 1, Pp 197-203 (2021)
Subject
Language
English
ISSN
2475-0379
Abstract
Abstract Background Sickle cell disease (SCD) is characterized by vaso‐occlusive crisis (VOC), acute chest syndrome (ACS) and multiorgan failure (MOF) complicated by thrombosis. Von Willebrand factor (VWF) is a strong marker of SCD‐related endothelial injury. Objectives To decipher the role of VWF and its specific‐cleaving metalloprotease, ADAMTS13, in the vaso‐occlusive and thrombotic process of SCD. Patients/Methods We investigated the VWF antigen (Ag), ADAMTS13 activity, ADAMTS13 Ag and ADAMTS13 IgGs in a cohort of 65 patients with SCD prospectively enrolled in a 20‐month period from three centers. Patients were divided into two groups: an asymptomatic group (n = 30) with treated or untreated SCD at steady state, and a VOC/ACS group (n = 35) with SCD with VOC/ACS requiring either medical management or intensive care management for MOF. Results and Conclusions VWF:Ag levels were increased (median, 167 IU/dL; interquartile range [IQR], 124 ‐ 279), especially in patients with VOC SCD (227 IU/dL; IQR, 134‐305; P = .04), and positively correlated with inflammatory markers (P