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Prepublished online as a Blood First Edition Paper on September 12, 2002; DOI 10.1182/blood-2002-03-0944.
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Blood, 1 February 2003, Vol. 101, No. 3, pp. 915-920
HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY
Mural thrombus generation in type 2A and 2B von Willebrand
disease under flow conditions
Mitsuhiko Sugimoto,
Hideto Matsui,
Tomohiro Mizuno,
Shizuko Tsuji,
Shigeki Miyata,
Masanori Matsumoto,
Michio Matsuda,
Yoshihiro Fujimura, and
Akira Yoshioka
From the Departments of Pediatrics and Blood
Transfusion, Nara Medical University, Kashihara, Japan;
the Division of Transfusion Medicine, National Cardiovascular Center,
Suita, Osaka, Japan; and the Center for Molecular
Medicine, Jichi Medical School, Kawachi-gun, Tochigi,
Japan.
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 s1),
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 s1, 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.
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