Affordable Access

deepdyve-link
Publisher Website

The rate of hemolysis in sickle cell disease correlates with the quantity of active von Willebrand factor in the plasma.

Authors
  • Chen, Junmei1
  • Hobbs, William E
  • Le, Jennie
  • Lenting, Peter J
  • de Groot, Philip G
  • López, José A
  • 1 Research Division, Puget Sound Blood Center, 921 Terry Avenue, Seattle, WA 98104, USA.
Type
Published Article
Journal
Blood
Publisher
American Society of Hematology
Publication Date
Mar 31, 2011
Volume
117
Issue
13
Pages
3680–3683
Identifiers
DOI: 10.1182/blood-2010-08-302539
PMID: 21300978
Source
Medline
License
Unknown

Abstract

Vaso-occlusion, hemolysis, and oxidative stress are hallmarks of sickle cell disease (SCD). This pathology is accompanied by systemic endothelial activation, rendering the endothelium more adhesive for blood cells, including sickle erythrocytes. Activated endothelial cells display or secrete several adhesive molecules, including von Willebrand factor (VWF). We assessed several VWF parameters in SCD patients at baseline: multimer pattern, antigen concentration (VWF:Ag), activation factor (VWF:AF), and total active VWF (VWF:TA). VWF:AF was determined using a llama nanobody (AU/VWFa-11) that detects a platelet-binding conformation of the A1 domain; VWF:TA was calculated by multiplying VWF:Ag by VWF:AF. SCD plasma contained elevated VWF:Ag and ultralarge VWF multimers. VWF:TA, a measure of total VWF reactivity, correlated closely with hemolysis, as determined by serum lactate dehydrogenase. ADAMTS13 activity and antigen were normal in all patients. These findings suggest an important role for hyperreactive VWF in SCD pathology and connect SCD to other microangiopathies, particularly thrombotic thrombocytopenic purpura.

Report this publication

Statistics

Seen <100 times