Thromb Haemost 2017; 117(08): 1528-1533
DOI: 10.1160/TH17-02-0129
Coagulation and Fibrinolysis
Schattauer GmbH

Whole blood ristocetin-activated platelet impedance aggregometry (Multiplate) for the rapid detection of Von Willebrand disease

David E Schmidt
1   Department of Medicine, Division of Haematology, Coagulation Unit, Karolinska University Hospital and Karolinska Institutet, Stockholm, Sweden
2   University Medical Center Utrecht, Utrecht, The Netherlands
,
Maria Bruzelius
1   Department of Medicine, Division of Haematology, Coagulation Unit, Karolinska University Hospital and Karolinska Institutet, Stockholm, Sweden
,
Ammar Majeed
1   Department of Medicine, Division of Haematology, Coagulation Unit, Karolinska University Hospital and Karolinska Institutet, Stockholm, Sweden
3   Department of Medical Epidemiology and Biostatistics, Karolinska Institutet, Stockholm, Sweden
,
Jacob Odeberg
1   Department of Medicine, Division of Haematology, Coagulation Unit, Karolinska University Hospital and Karolinska Institutet, Stockholm, Sweden
,
Margareta Holmström
1   Department of Medicine, Division of Haematology, Coagulation Unit, Karolinska University Hospital and Karolinska Institutet, Stockholm, Sweden
,
Anna Ågren
1   Department of Medicine, Division of Haematology, Coagulation Unit, Karolinska University Hospital and Karolinska Institutet, Stockholm, Sweden
› Author Affiliations
Further Information

Publication History

Received: 22 February 2017

Accepted after minor revision: 09 May 2017

Publication Date:
22 November 2017 (online)

Summary

Von Willebrand disease (VWD) is the most common bleeding disorder, but no bedside tests specific for Von Willebrand factor are available. The objective of this study was to evaluate the diagnostic accuracy of whole blood ristocetin-induced platelet aggregometry (WB-RIPA) in VWD. WB-RIPA was performed in VWD patients (n=100) and healthy controls (n=17) using the Multiplate® platelet impedance aggregometry platform. The diagnostic properties of the test were described as sensitivity/specificity, positive and negative predictive value, and ROC area under the curve (AUC). Patients with VWD had impaired platelet aggregation by WB-RIPA. At a cut-off of 98 U, the test sensitivity and specificity of WB-RIPA for VWD was 0.95 and 0.53. A cut-off of 60 U provided a specificity of 1.00 with reduced sensitivity of 0.76. All patients with type 3 VWD and >90% of patients with type 2 VWD were accurately distinguished from the controls. Incorrect classifications were attributable to patients with type 1 VWD, showing partly overlapping WB-RIPA results with healthy controls. Remarkably, these patients had lower bleeding scores and higher VWF activity than other type 1 VWD patients. Overall, WB-RIPA discriminated VWD patients from healthy controls accurately with a ROC AUC of 0.94. These results show that WB-RIPA is a promising diagnostic test for VWD, especially when timely results are required. Depending on the chosen test threshold, WB-RIPA could be clinically used as a rule out test, or to suggest patients in whom further testing for VWD is warranted.

 
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