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von Willebrand Disease: Diagnosis and Treatment

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Recent Advances in Thrombosis and Hemostasis 2008

Abstract

von Willebrand disease (VWD) is caused by quantitative or qualitative defects of von Willebrand factor (VWF), associated with mucocutaneous bleeding symptoms. VWD is classified into three major groups and four subcategories. The classification is intended to be simple, to rely on widely available laboratory tests, and to correlate with important clinical characteristics. It is meant to facilitate the diagnosis, treatment, and counseling of patients with VWD. The International Society on Thrombosis and Haemostasis/Scientific and Standardization Committee (ISTH/SSC) reviewed the classification and published update information on the diagnosis and treatment of VWD in 2006 based on the most recent pathophysiology data for VWF. Notably, Vicenza type has been classified into type 1 from type 2M. In epidemiology, there may be small differences of frequency in VWD subcategories (type 2) between Europe and lapan. For treatment, factor VIII/VWF concentrate is commonly employed in Japan, although DDAVP has been the first choice for VWD in Europe.

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Authors and Affiliations

  1. Nara Prefectural Mimuro Hospital, 1-14-16 Mimuro, Sango-cho, Ikoma-gun, Nara, 636-0802, Japan

    Masato Nishino

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  1. Masato Nishino
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Editors and Affiliations

  1. Department of Pathology, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan

    Kenzo Tanaka M.D., Ph.D. (Professor Emeritus) (Professor Emeritus)

  2. Department of Biochemistry, University of Washington, J317 Health Science Center, Box 357350, Seattle, WA, 98195, USA

    Earl W. Davie Ph.D. (Professor) (Professor)

  3. School of Medicine, Internal Medicine, Keio University, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan

    Yasuo Ikeda M.D., Ph.D. (Professor) (Professor)

  4. Department of Biology, Kyushu University, 6-10-1 Hakozaki, Higashi-ku, Fukuoka, 812-8581, Japan

    Sadaaki Iwanaga Ph.D. (Professor Emeritus) (Professor Emeritus)

  5. Department of Medicine, Nagoya University, 26 Tsurumai-cho, Showa-ku, Nagoya, 466-8550, Japan

    Hidehiko Saito M.D., Ph.D. (Professor Emeritus) (Professor Emeritus)

  6. Pathophysiological and Experimental Division, Department of Pathology, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan

    Katsuo Sueishi M.D., Ph.D. (Professor) (Professor)

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Nishino, M. (2008). von Willebrand Disease: Diagnosis and Treatment. In: Tanaka, K., Davie, E.W., Ikeda, Y., Iwanaga, S., Saito, H., Sueishi, K. (eds) Recent Advances in Thrombosis and Hemostasis 2008. Springer, Tokyo. https://doi.org/10.1007/978-4-431-78847-8_29

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  • DOI: https://doi.org/10.1007/978-4-431-78847-8_29

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  • Online ISBN: 978-4-431-78847-8

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