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, G.W. MOORE and A. GADISSEUR. A comparative analysis of different automated von Willebrand factor glycoprotein Ib-binding activity assays in well typed von Willebrand disease patients. Journal of Thrombosis and Haemostasis. HOBOKEN: WILEY, 2018, vol. 16, No 7, p. 1268-1277. ISSN 1538-7933. Available from: https://dx.doi.org/10.1111/jth.14145.

Basic information

• Original name: A comparative analysis of different automated von Willebrand factor glycoprotein Ib-binding activity assays in well typed von Willebrand disease patients
• Authors: VANGENECHTEN, I. (56 Belgium, guarantor), K. MAYGER (826 United Kingdom of Great Britain and Northern Ireland), Petr SMEJKAL (203 Czech Republic, belonging to the institution), Ondřej ZAPLETAL (203 Czech Republic), J.J. MICHIELS (56 Belgium), G.W. MOORE (826 United Kingdom of Great Britain and Northern Ireland) and A. GADISSEUR (56 Belgium).
• Edition: Journal of Thrombosis and Haemostasis, HOBOKEN, WILEY, 2018, 1538-7933.

Other information

• Original language: English
• Type of outcome: Article in a journal
• Field of Study: 30205 Hematology
• Country of publisher: United States of America
• Confidentiality degree: is not subject to a state or trade secret
• Impact factor: Impact factor: 4.662
• RIV identification code: RIV/00216224:14110/18:00104167
• Organization unit: Faculty of Medicine
• Doi: http://dx.doi.org/10.1111/jth.14145
• UT WoS: 000437289500005
• Keywords in English: classification; ristocetin cofactor; subtypes; Von Willebrand disease; von Willebrand factor
• Tags: 14110616, rivok
• Tags: International impact, Reviewed

Abstract

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.