A comparative analysis of different automated von Willebrand factor glycoprotein Ib-binding activity assays in well typed von Willebrand disease patients
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F65269705%3A_____%2F18%3A00069284" target="_blank" >RIV/65269705:_____/18:00069284 - isvavai.cz</a>
Alternative codes found
RIV/00216224:14110/18:00104167
Result on the web
<a href="http://dx.doi.org/10.1111/jth.14145" target="_blank" >http://dx.doi.org/10.1111/jth.14145</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1111/jth.14145" target="_blank" >10.1111/jth.14145</a>
Alternative languages
Result language
angličtina
Original language name
A comparative analysis of different automated von Willebrand factor glycoprotein Ib-binding activity assays in well typed von Willebrand disease patients
Original language description
Background: von Willebrand disease (VWD) is an inherited bleeding disorder caused by quantitative (type 1 and 3) or qualitative (type 2) von Willebrand factor (VWF) defect. VWD diagnosis and classification require numerous laboratory tests. VWF: glycoprotein Ib (GPIb)-binding activity assays are used to distinguish type 1 from type 2 VWD. Objectives: Three different automated VWF:GPIb-binding activity assays were compared. Patients and methods: BC-VWF:RCo (Siemens Healthcare Diagnostics), HemosIL((R)) VWF:RCo (Instrumentation Laboratory) and INNOVANCE((R)) VWF:Ac (Siemens Healthcare Diagnostics) were performed in a well typed VWD cohort (n = 142). Results: Based on the three most used VWD parameters (FVIII:C, VWF:Ag and VWF:GPIb-binding activity) and using a cut-off of <0.70 for type 2 VWD revealed sensitivity and specificity of, respectively, 92% and 72.4% for VWF:RCo/VWF:Ag, 84% and 89.7% for VWF:GPIbR/VWF:Ag, and 92% and 85.1% for VWF:GPIbM/VWF:Ag, whereas a lowered cut-off of < 0.60 resulted in reduced sensitivity with increased specificity for all assays. Conclusion: VWD classification based on FVIII:C, VWF:Ag and VWF:GPIb-binding activity revealed an overall problem with normal VWF:GPIb-binding activity/VWF:Ag within type 2, especially type 2A/IIE. Although all assays were practically identical, BC-VWF:RCo had higher %CV compared with both new assays but comparable lower limit of quantification (LLOQ) similar to 4 IU dL(-1). No clear improved distinction between type 1 and 2 VWD with new assays was seen. BC-VWF:RCo and HemosIL((R)) are ristocetin dependent, whereas INNOVANCE((R)) does not rely upon ristocetin and is not influenced by VWF polymorphisms increasing VWF:GPIb-binding activity levels. INNOVANCE((R)) seems to be the best choice as a first-line VWF:GPIb-binding activity assay, providing the best balance between sensitivity and specificity for type 2 VWD.
Czech name
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Czech description
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Classification
Type
J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database
CEP classification
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OECD FORD branch
30205 - Hematology
Result continuities
Project
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Continuities
I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace
Others
Publication year
2018
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
Journal of thrombosis and haemostasis
ISSN
1538-7933
e-ISSN
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Volume of the periodical
16
Issue of the periodical within the volume
7
Country of publishing house
US - UNITED STATES
Number of pages
10
Pages from-to
1268-1277
UT code for WoS article
000437289500005
EID of the result in the Scopus database
2-s2.0-85049500253