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Whole blood ristocetin‐induced platelet impedance aggregometry does not reflect clinical severity in patients with type 1 von Willebrand disease

Authors :
Yuto Nakajima
Shoko Furukawa
Koji Yada
Naruto Shimonishi
Midori Shima
Keiji Nogami
Kenichi Ogiwara
Source :
Haemophilia. 25
Publication Year :
2019
Publisher :
Wiley, 2019.

Abstract

The 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.To investigate the relationship between WB-RIPA, bleeding scores (BS) and VWF-related measurements in type 1 VWD.WB-RIPA assay using the Multiplate® was performed using whole blood from 55 patients with type 1 VWD. BS was determined using a standardized questionnaire.WB-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 BS4 (defined as no abnormal bleeding tendency), respectively. However, no significant difference was observed in WB-RIPA between the BS ≥4 group and BS4 group. Overall, VWD patients with a WB-RIPA level70 U did not seem to have an abnormal bleeding tendency, but low levels of WB-RIPA did not correlate with BS.WB-RIPA did not reflect clinical severity in type 1 VWD patients.

Details

ISSN :
13652516 and 13518216
Volume :
25
Database :
OpenAIRE
Journal :
Haemophilia
Accession number :
edsair.doi.dedup.....b9d96b704541fdac0f14b2f73db35a7e
Full Text :
https://doi.org/10.1111/hae.13725