Mural thrombus generation in type 2A and 2B von Willebrand disease under flow conditions
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- Mitsuhiko Sugimoto
- 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.
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- Hideto Matsui
- 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.
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- Tomohiro Mizuno
- 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.
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- Shizuko Tsuji
- 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.
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- Shigeki Miyata
- 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.
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- Masanori Matsumoto
- 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.
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- Michio Matsuda
- 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.
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- Yoshihiro Fujimura
- 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.
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- 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.
書誌事項
- 公開日
- 2003-02-01
- DOI
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- 10.1182/blood-2002-03-0944
- 公開者
- American Society of Hematology
この論文をさがす
説明
<jats:title>Abstract</jats:title><jats:p>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.</jats:p>
収録刊行物
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- Blood
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Blood 101 (3), 915-920, 2003-02-01
American Society of Hematology
