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Whole blood ristocetin‐induced platelet impedance aggregometry does not reflect clinical severity in patients with type 1 von Willebrand disease
- 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.
- Subjects :
- Adult
Male
congenital, hereditary, and neonatal diseases and abnormalities
medicine.medical_specialty
Adolescent
Platelet Aggregation
030204 cardiovascular system & hematology
Severity of Illness Index
von Willebrand Disease, Type 1
Gastroenterology
Young Adult
03 medical and health sciences
chemistry.chemical_compound
0302 clinical medicine
Von Willebrand factor
hemic and lymphatic diseases
Internal medicine
von Willebrand Factor
Electric Impedance
Von Willebrand disease
Humans
Medicine
Platelet
In patient
Ristocetin
Genetics (clinical)
Whole blood
biology
business.industry
Abnormal bleeding
Hematology
General Medicine
Middle Aged
medicine.disease
Agglutination (biology)
Phenotype
chemistry
Case-Control Studies
biology.protein
Female
business
030215 immunology
Subjects
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