Clinical Features of Our Patients with von Willebrand's Disease

DOI Web Site PubMed
  • TAKAHASHI Hoyu
    First Department of Internal Medicine, Niigata University School of Medicine
  • KOIKE Kazuo
    First Department of Internal Medicine, Niigata University School of Medicine
  • ITOH Masakazu
    First Department of Internal Medicine, Niigata University School of Medicine
  • HATTORI Akira
    First Department of Internal Medicine, Niigata University School of Medicine
  • KOBAYASHI Isao
    First Department of Internal Medicine, Niigata University School of Medicine
  • TAKAHASHI Kaoru
    First Department of Internal Medicine, Niigata University School of Medicine
  • SAKURAGAWA Nobuo
    First Department of Internal Medicine, Niigata University School of Medicine
  • TSUKADA Tsuneyasu
    Division of Hematology, Niigata Shimin Hospital
  • SHIBATA Akira
    First Department of Internal Medicine, Niigata University School of Medicine
  • MATSUOKA Matsuzo
    First Department of Internal Medicine, Niigata University School of Medicine

Bibliographic Information

Other Title
  • von Willebrand病自験例の臨床所見
  • von Willebrandビョウ ジケンレイ ノ リンショウ ショケン

Search this article

Description

The clinical features of 47 patients with von Willebrand's disease were studied. Nose bleeding was the most common symptom and ecchymoses, bleeding after tooth extraction, gingival bleeding and prolonged bleedings from trivial wounds were fairly common. Gynecological bleedings were also prominent symptoms in females. One patient revealed a joint bleeding with arthropathy and one patient showed an intramuscular bleeding. The mode of inheritance was mainly autosomal dominant, but there were five sporadic cases. The parental consanguinity was recongnized in three families.<br>Von Willebrand factor activity (VIII R: WF) appeared to be the most useful parameter to diagnose von Willebrand's disease. There were five patients with undetectable factor VIII related antigen (VIII R: AG), but VIII R: AG and factor V III procoagulant activity (VIII: C) were within the normal range in a number of patients. An increased anodal migration of VIII R: AG was detected in 14 of 18 patients in which was performed two-dimensional crossed immunoelectophoresis. Among classical cases there were many patients who showed increased electrophoretic mobility of VIIIR: AG.<br>There was a significant positive correlation between VIII R: AG and VIII: C (r=0.710, p<0.001) and the ratio of VIII: C/VIII R: AG was more than 1.0 in patients with decreased VIII R: AG. There was no significant correlation between VIII: C and VIIIR: WF. Although there was a positive correlation between VIII R: AG and VIII R: WF (r=0.407, p<0.02), there was a discrepancy between them in patients with normal VIII R: AG. This finding suggested that there was a qualitative abnormality of the factor VIII molecules in these patients.<br>It was assumed that patients with von Willebrand's disease had a defect in primary hemostasis with reduced VIII R: WF due to a quantitative and/or qualitative abnormality of the factor VIII protein.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 21 (2), 179-188, 1980

    The Japanese Society of Hematology

Keywords

Details 詳細情報について

Report a problem

Back to top