Analysis of College of American Pathologists von Willebrand Factor Proficiency Testing Program

Abstract

Von Willebrand factor (VWF) level and/or function is altered in von Willebrand disease (VWD), the most common heritable bleeding disorder worldwide. Laboratory assessment of VWF is continually evolving. Historically, the primary method for the assessment of VWF platelet-binding activity was the ristocetin cofactor assay (VWF:RCo). Contemporary alternative measures of VWF platelet-binding activity include VWF:GPIbR (recombinant; using ristocetin), VWF:GPIbM (recombinant; gain-of-function mutant), and monoclonal antibody. Recently, the American Society of Hematology, International Society on Thrombosis and Haemostasis, National Hemophilia Foundation, and World Federation of Hemophilia collaboration issued guidelines recommending the use of newer assays of VWF platelet-binding activity (VWF: GPIbM, VWF: GPIbR) over VWF:RCo, given known limitations of the VWF:RCo assay. Despite this recommendation, the newer VWF:GPIbM and VWF:GPIbR assays are not United States Food and Drug Administration cleared, limiting their availability in the United States. We sought to assess assay utilization trends, agreement of VWF testing methods, and imprecision of VWF testing (based on assigned sample type) from the College of American Pathologists Proficiency Testing Surveys. The analysis confirms that, while VWF antigen testing has low imprecision, the various VWF activity assays have significant interassay variability, with VWF:RCo showing greater imprecision than the newer GPIb-binding assays. The overall trends in assay utilization reflect the barriers to complete compliance with modern VWD diagnostic guidelines in North America.

Keywords

von Willebrand disease - coagulation special testing - proficiency testing

Publikationsverlauf

Artikel online veröffentlicht:
02. September 2022

• References

• 1 Sadler JE, Budde U, Eikenboom JC. et al; Working Party on von Willebrand Disease Classification. Update on the pathophysiology and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand Factor. J Thromb Haemost 2006; 4 (10) 2103-2114
• 2 Langer AL, Connell NT. Acquired von Willebrand Syndrome. Hematol Oncol Clin North Am 2021; 35 (06) 1103-1116
• 3 Rodeghiero F, Castaman G, Dini E. Epidemiological investigation of the prevalence of von Willebrand's disease. Blood 1987; 69 (02) 454-459 (In Eng)
• 4 Favaloro EJ. Commentary on “ASH ISTH NHF WFH 2021 guidelines on the diagnosis of VWD”: reflections based on recent contemporary test data. Blood Adv 2022; 6 (02) 416-419
• 5 Howard MA, Firkin BG. Ristocetin—a new tool in the investigation of platelet aggregation. Thromb Diath Haemorrh 1971; 26 (02) 362-369 (In Eng)
• 6 Olson JD, Brockway WJ, Fass DN, Magnuson MA, Bowie EJ. Evaluation of ristocetin-Willebrand factor assay and ristocetin-induced platelet aggregation. Am J Clin Pathol 1975; 63 (02) 210-218
• 7 Favaloro EJ, Dean E, Arunachalam S, Vong R, Mohammed S. Evaluating errors in the laboratory identification of von Willebrand disease using contemporary von Willebrand factor assays. Pathology 2022; 54 (03) 308-317
• 8 Flood VH, Gill JC, Morateck PA. et al. Common VWF exon 28 polymorphisms in African Americans affecting the VWF activity assay by ristocetin cofactor. Blood 2010; 116 (02) 280-286
• 9 Chen D, Tange JI, Meyers BJ, Pruthi RK, Nichols WL, Heit JA. Validation of an automated latex particle-enhanced immunoturbidimetric von Willebrand factor activity assay. J Thromb Haemost 2011; 9 (10) 1993-2002
• 10 James PD, Connell NT, Ameer B. et al. ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease. Blood Adv 2021; 5 (01) 280-300
• 11 Patzke J, Budde U, Huber A. et al. Performance evaluation and multicentre study of a von Willebrand factor activity assay based on GPIb binding in the absence of ristocetin. Blood Coagul Fibrinolysis 2014; 25 (08) 860-870
• 12 Abdulrehman J, Ziemba YC, Hsu P. et al. Diagnosis of von Willebrand disease: an assessment of the quality of testing in North American laboratories. Haemophilia 2021; 27 (06) e713-e720
• 13 Stufano F, Baronciani L, Bucciarelli P. et al. Evaluation of a fully automated von Willebrand factor assay panel for the diagnosis of von Willebrand disease. Haemophilia 2020; 26 (02) 298-305
• 14 Pikta M, Szanto T, Viigimaa M. et al. Evaluation of a new semi-automated Hydragel 11 von Willebrand factor multimers assay kit for routine use. J Med Biochem 2021; 40 (02) 167-172
• 15 Gardiner C, Coleman R, de Maat MPM. et al. International Council for Standardization in Haematology (ICSH) laboratory guidance for the verification of haemostasis analyser-reagent test systems. Part 2: specialist tests and calibrated assays. Int J Lab Hematol 2021; 43 (05) 907-916
• 16 Favaloro EJ, Bonar RA, Mohammed S. et al. Type 2M von Willebrand disease - more often misidentified than correctly identified. Haemophilia 2016; 22 (03) e145-e155