• Yuto Nakajima, Keiji Nogami, Koji Yada, Kenichi Ogiwara, Shoko Furukawa, Naruto Shimonishi, and Midori Shima.
• Department of Pediatrics, Nara Medical University, Kashihara, Nara, Japan.
• Haemophilia. 2019 May 1; 25 (3): e174-e179.

BackgroundThe haemorrhagic phenotype in patients with von Willebrand disease (VWD) is heterogeneous, and assays of von Willebrand factor ristocetin cofactor activity (VWF:RCo) do not always reflect clinical severity, especially in those individuals classed as type 1 VWD. Recent studies have shown that whole blood ristocetin-induced platelet agglutination (WB-RIPA) using an easy-to-use analyzer, Multiplate® platelet impedance technique, could be informative as a diagnostic test in VWD, although inconsistencies were evident in patients with the type 1 disorder, possibly associated with clinical symptoms.AimTo investigate the relationship between WB-RIPA, bleeding scores (BS) and VWF-related measurements in type 1 VWD.MethodsWB-RIPA assay using the Multiplate® was performed using whole blood from 55 patients with type 1 VWD. BS was determined using a standardized questionnaire.ResultsWB-RIPA values were significantly lower in type 1 VWD than in healthy controls (P < 0.0001). Weak correlations were apparent between WB-RIPA and VWF:RCo or VWF antigen (VWF:Ag; r = 0.22 or 0.28, respectively). There were significant differences in VWF:RCo (P = 0.036) and VWF:Ag (P = 0.0013) between patients with BS ≥4 (defined as abnormal bleeding tendency) and BS <4 (defined as no abnormal bleeding tendency), respectively. However, no significant difference was observed in WB-RIPA between the BS ≥4 group and BS <4 group. Overall, VWD patients with a WB-RIPA level >70 U did not seem to have an abnormal bleeding tendency, but low levels of WB-RIPA did not correlate with BS.ConclusionWB-RIPA did not reflect clinical severity in type 1 VWD patients.© 2019 John Wiley & Sons Ltd.

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