TY - CHAP
T1 - Laboratory testing for von Willebrand disease using a composite rapid 3-test chemiluminescence-based von Willebrand factor assay panel
AU - Favaloro, Emmanuel J
AU - Mohammed, Soma
AU - Vong, Ronny
AU - Pasalic, Leonardo
N1 - Publisher Copyright:
© 2023, The Author(s), under exclusive license to Springer Science+Business Media, LLC, part of Springer Nature.
PY - 2023
Y1 - 2023
N2 - von Willebrand disease (VWD) is the most commonly reported inherited bleeding disorder and may alternatively occur as an acquired von Willebrand syndrome (AVWS). VWD/AVWS develops from defects and/or deficiency in the adhesive plasma protein von Willebrand factor (VWF). VWD/AVWS diagnosis/exclusion remains challenging because of the heterogeneity of VWF defects and the technical limitations of many VWF tests, as well as the VWF test panels (number and type of tests) chosen by many laboratories. Laboratory testing for these disorders utilizes evaluation of VWF level and activity, with activity assessment needing several tests due to the many functions performed by VWF in order to help counteract bleeding. This report explains procedures for evaluating VWF level (antigen; VWF:Ag) and activity by means of a chemiluminescence-based panel. Activity assays comprise collagen binding (VWF:CB) and a ristocetin-based recombinant glycoprotein Ib-binding (VWF:GPIbR) assay that reflects a contemporary alternative to classical ristocetin cofactor (VWF:RCo). This 3-test VWF panel (Ag, CB, GPIbR [RCo]) reflects the only such composite panel available on a single platform and is performed on an AcuStar instrument (Werfen/Instrumentation Laboratory). Certain regional approvals may also allow this 3-test VWF panel to be performed on the BioFlash instrument (Werfen/Instrumentation Laboratory).
AB - von Willebrand disease (VWD) is the most commonly reported inherited bleeding disorder and may alternatively occur as an acquired von Willebrand syndrome (AVWS). VWD/AVWS develops from defects and/or deficiency in the adhesive plasma protein von Willebrand factor (VWF). VWD/AVWS diagnosis/exclusion remains challenging because of the heterogeneity of VWF defects and the technical limitations of many VWF tests, as well as the VWF test panels (number and type of tests) chosen by many laboratories. Laboratory testing for these disorders utilizes evaluation of VWF level and activity, with activity assessment needing several tests due to the many functions performed by VWF in order to help counteract bleeding. This report explains procedures for evaluating VWF level (antigen; VWF:Ag) and activity by means of a chemiluminescence-based panel. Activity assays comprise collagen binding (VWF:CB) and a ristocetin-based recombinant glycoprotein Ib-binding (VWF:GPIbR) assay that reflects a contemporary alternative to classical ristocetin cofactor (VWF:RCo). This 3-test VWF panel (Ag, CB, GPIbR [RCo]) reflects the only such composite panel available on a single platform and is performed on an AcuStar instrument (Werfen/Instrumentation Laboratory). Certain regional approvals may also allow this 3-test VWF panel to be performed on the BioFlash instrument (Werfen/Instrumentation Laboratory).
KW - Glycoprotein Ib binding
KW - Ristocetin cofactor
KW - von Willebrand disease
KW - von Willebrand factor (VWF)
KW - von Willebrand factor collagen binding (VWF:CB)
KW - VWD
KW - VWF:GPIbR
KW - VWF:RCo
UR - http://www.scopus.com/inward/record.url?scp=85159762717&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85159762717&partnerID=8YFLogxK
U2 - 10.1007/978-1-0716-3175-1_43
DO - 10.1007/978-1-0716-3175-1_43
M3 - Chapter (peer-reviewed)
C2 - 37204743
SN - 9781071631744
T3 - Methods in Molecular Biology
SP - 647
EP - 667
BT - Hemostasis and thrombosis
A2 - , Emmanuel J. Favaloro
A2 - , Robert C. Gosselin
PB - Humana Press
CY - New York
ER -