Document Detail

Mural thrombus generation in type 2A and 2B von Willebrand disease under flow conditions.
MedLine Citation:
PMID:  12393671     Owner:  NLM     Status:  MEDLINE    
To explore the mechanisms that underlie the bleeding tendency in type 2A and 2B von Willebrand disease (VWD), we analyzed the mural thrombus generation process on a collagen surface under physiologic blood flow in a perfusion chamber using whole blood from these VWD patients. At a low shear rate (50 s(-1)), thrombus generation in all type 2A and 2B VWD patients was comparable to that of healthy controls. At a high shear rate (1500 s(-1)), thrombus generation was impaired in all type 2A patients, whereas that in type 2B VWD patients varied from normal to significantly defective, as judged by epifluorescence microscopy of thrombus surface coverage. However, in type 2B patients who showed normal thrombus generation at 1500 s(-1), the height and volume of thrombi was significantly reduced, albeit with the normal surface coverage, compared with control thrombi, and von Willebrand factor (VWF) was poorly distributed within the type 2B thrombus mass when analyzed in detail by confocal laser scanning microscopy. Addition of purified VWF to patient blood completely reversed the defective spatial thrombus growth in type 2B VWD. Thus, our results confirm the impaired thrombus generation in type 2B VWD, which has never been demonstrable in previous in vitro soluble-phase platelet aggregation assays, and point to the critical function of larger VWF multimers in the proper spatial growth of mural thrombi under high shear rate conditions.
Mitsuhiko Sugimoto; Hideto Matsui; Tomohiro Mizuno; Shizuko Tsuji; Shigeki Miyata; Masanori Matsumoto; Michio Matsuda; Yoshihiro Fujimura; Akira Yoshioka
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2002-09-12
Journal Detail:
Title:  Blood     Volume:  101     ISSN:  0006-4971     ISO Abbreviation:  Blood     Publication Date:  2003 Feb 
Date Detail:
Created Date:  2003-01-16     Completed Date:  2003-04-21     Revised Date:  2009-11-19    
Medline Journal Info:
Nlm Unique ID:  7603509     Medline TA:  Blood     Country:  United States    
Other Details:
Languages:  eng     Pagination:  915-20     Citation Subset:  AIM; IM    
Department of Pediatrics, Nara Medical University, Kashihara, Japan.
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MeSH Terms
Blood Coagulation / drug effects
Case-Control Studies
Family Health
Heart Diseases / blood*
Hemorrhage / etiology*
Microscopy, Fluorescence
Models, Cardiovascular
Thrombosis / blood*
von Willebrand Diseases / blood,  classification,  complications*
von Willebrand Factor / metabolism,  pharmacology
Reg. No./Substance:
0/von Willebrand Factor; 9007-34-5/Collagen

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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