J 2018

A comparative analysis of different automated von Willebrand factor glycoprotein Ib-binding activity assays in well typed von Willebrand disease patients

VANGENECHTEN, I., K. MAYGER, Petr SMEJKAL, Ondřej ZAPLETAL, J.J. MICHIELS et. al.

Základní údaje

Originální název

A comparative analysis of different automated von Willebrand factor glycoprotein Ib-binding activity assays in well typed von Willebrand disease patients

Autoři

VANGENECHTEN, I. (56 Belgie, garant), K. MAYGER (826 Velká Británie a Severní Irsko), Petr SMEJKAL (203 Česká republika, domácí), Ondřej ZAPLETAL (203 Česká republika), J.J. MICHIELS (56 Belgie), G.W. MOORE (826 Velká Británie a Severní Irsko) a A. GADISSEUR (56 Belgie)

Vydání

Journal of Thrombosis and Haemostasis, HOBOKEN, WILEY, 2018, 1538-7933

Další údaje

Jazyk

angličtina

Typ výsledku

Článek v odborném periodiku

Obor

30205 Hematology

Stát vydavatele

Spojené státy

Utajení

není předmětem státního či obchodního tajemství

Impakt faktor

Impact factor: 4.662

Kód RIV

RIV/00216224:14110/18:00104167

Organizační jednotka

Lékařská fakulta

DOI

UT WoS

000437289500005

Klíčová slova anglicky

classification; ristocetin cofactor; subtypes; Von Willebrand disease; von Willebrand factor

Štítky

Příznaky

Mezinárodní význam, Recenzováno
Změněno: 2. 5. 2019 14:17, Soňa Böhmová

Anotace

V originále

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.