Essentials Von Willebrand ristocetin cofactor activity (VWF:RCo) is not a completely reliable assay. Three automated VWF activity assays were compared within a von Willebrand disease (VWD) cohort. Raw values for all three assays were virtually the same. An overall problem within type 2A/IIE VWD using VWF:GPIb-binding activity/VWF:Ag was observed. SUMMARY: 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® VWF:RCo (Instrumentation Laboratory) and INNOVANCE® 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) ~4 IU dL-1 . No clear improved distinction between type 1 and 2 VWD with new assays was seen. BC-VWF: RCo and HemosIL® are ristocetin dependent, whereas INNOVANCE® does not rely upon ristocetin and is not influenced by VWF polymorphisms increasing VWF:GPIb-binding activity levels. INNOVANCE® 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.
- MeSH
- biologické markery krev MeSH
- design vybavení MeSH
- hematologické testy přístrojové vybavení metody MeSH
- laboratorní automatizace MeSH
- lidé MeSH
- prediktivní hodnota testů MeSH
- průřezové studie MeSH
- reprodukovatelnost výsledků MeSH
- trombocytový glykoproteinový komplex Ib-IX metabolismus MeSH
- vazba proteinů MeSH
- von Willebrandova nemoc krev klasifikace diagnóza MeSH
- von Willebrandův faktor metabolismus MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- multicentrická studie MeSH
- srovnávací studie MeSH
- Geografické názvy
- Belgie MeSH
- Česká republika MeSH