Treatment of von Willebrand disease

Research output: Contribution to journalArticle

28 Citations (Scopus)

Abstract

von Willebrand disease is the most frequent of inherited bleeding disorders (1:100 affected individuals in the general population). The aim of treatment is to correct the dual defects of haemostasis, i.e., abnormal coagulation expressed by low levels of factor VIII and abnormal platelet adhesion expressed by a prolonged bleeding time. There are two main options available for the management of von Willebrand disease: desmopressin and transfusion therapy with blood products. Desmopressin is the treatment of choice in patients with type 1 von Willebrand disease, who account for approximately 80% of cases. This pharmacological compound raises endogenous factor VIII and von Willebrand factors and thereby corrects the intrinsic coagulation defect and the prolonged bleeding time in most type 1 patients. In type 3 and in the majority of type 2 patients desmopressin is not effective, and it is necessary to resort to plasma concentrates containing factor VIII and von Willebrand factor. Treated with virucidal methods, these concentrates are effective and currently safe, but the bleeding time defect is not always corrected by them. Platelet concentrates or desmopressin can be used as adjunctive treatments when poor correction of the bleeding time after concentrates is associated with continued bleeding.

Original languageEnglish
Pages (from-to)661-664
Number of pages4
JournalHaemophilia
Volume4
Issue number4
Publication statusPublished - 1998

Fingerprint

von Willebrand Diseases
Deamino Arginine Vasopressin
Bleeding Time
Blood Platelets
Type 1 von Willebrand Disease
Hemorrhage
Factor VIII
Therapeutics
Hemostasis
Pharmacology
Population
factor VIII, von Willebrand factor drug combination

Keywords

  • Desmopressin
  • Factor VIII
  • Haemophilia
  • von Willebrand disease
  • von Willebrand factor

ASJC Scopus subject areas

  • Hematology

Cite this

Treatment of von Willebrand disease. / Mannucci, P. M.

In: Haemophilia, Vol. 4, No. 4, 1998, p. 661-664.

Research output: Contribution to journalArticle

Mannucci, PM 1998, 'Treatment of von Willebrand disease', Haemophilia, vol. 4, no. 4, pp. 661-664.
Mannucci, P. M. / Treatment of von Willebrand disease. In: Haemophilia. 1998 ; Vol. 4, No. 4. pp. 661-664.
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