Comprehensive evaluation of haemostatic function in von Willebrand disease patients using a microchip-based flow chamber system

Summary The diagnosis of von Willebrand disease (VWD) is difficult due to the wide spectrum of clinical phenotypes associated with this disorder. We have analysed and characterized haemostatic function in VWD patients using a microchip‐based flow chamber system. Microchips coated with either collage...

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Bibliographic Details
Published in:Haemophilia : the official journal of the World Federation of Hemophilia 2015-01, Vol.21 (1), p.71-80
Main Authors: Ogiwara, K., Nogami, K., Hosokawa, K., Ohnishi, T., Matsumoto, T., Shima, M.
Format: Article
Language:English
Subjects:
clinical phenotype
Deamino Arginine Vasopressin - therapeutic use
desmopressin
Drug Combinations
factor VIII
Factor VIII - therapeutic use
Female
flow chamber system
Hemostasis
Hemostatics - therapeutic use
Humans
von Willebrand disease
von Willebrand Diseases - blood
von Willebrand factor
von Willebrand Factor - therapeutic use
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Summary:Summary The diagnosis of von Willebrand disease (VWD) is difficult due to the wide spectrum of clinical phenotypes associated with this disorder. We have analysed and characterized haemostatic function in VWD patients using a microchip‐based flow chamber system. Microchips coated with either collagen [platelet (PL)‐chip] or collagen/thromboplastin [atherome (AR)‐chip] were used to evaluate platelet thrombus formation at 1000 s−1 and fibrin‐rich platelet thrombus formation at 240 s−1 respectively. Blood samples from an asymptomatic patient with VWD type 1 [von Willebrand factor (VWF): RCo 3.2%; bleeding score (BS 2] displayed normal thrombus formation in both PL‐ and AR‐chips, whereas blood from a symptomatic type 1 patient (VWF: RCo 14%, BS 9) had significantly delayed capillary occlusion. Nearly complete suppression of the flow pressure increase was observed in symptomatic patients with VWD type 2A (BS 13) and 2N (BS 27), whereas no flow pressure was found for the type 3 patient (BS 6). Fibrin‐rich platelet thrombus formation was only weakly increased by the in vitro addition of factor VIII (FVIII) to blood samples from the type 3 patient, but was normalized by the addition of VWF/FVIII. The in vivo effects of treatment with desmopressin or VWF/FVIII for the symptomatic patients were analysed using two types of microchips. The PL‐chip was highly sensitive for patients’ VWF‐mediated platelet functions, whereas the AR‐chip allowed assessment of overall haemostatic ability, including sensitivity to both VWF and FVIII. The combined analysis with PL‐ and AR‐chips may be potentially useful for the diagnosis of VWD based on clinical phenotypes, and for monitoring drug effects.
ISSN:1351-8216
1365-2516
DOI:10.1111/hae.12610