Management of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctors' Organization

Abstract

Summary. von Willebrand disease (VWD) is the commonest inherited bleeding disorder. The aim of therapy for VWD is to correct the two defects of haemostasis in this disorder, impaired primary haemostasis because of defective platelet adhesion and aggregation and impaired coagulation as a result of low levels of factor VIII. The objective of this guideline is to inform individuals making choices about the treatment and management of VWD including the use of therapeutic products. This is the second edition of this UK Haemophilia Centre Doctors' Organization (UKHCDO) guideline and supersedes the previous edition which was published in 1994.

The obstetric and gynaecological management of women with inherited bleeding disorders – review with guidelines produced by a taskforce of UK Haemophilia Centre Doctors’ Organization

“…DDAVP is not contraindicated in uncomplicated pregnancy although like all drugs it should be used with caution. Prolonged administration in pregnancy should be avoided and close monitoring for water retention is important [67]. A fluid restriction of 1 L for 24 h after DDAVP should minimize the risk of fluid overload and consequent hyponatraemia.…”

“…In all cases the epidural should be inserted by an experienced anaesthetist. Epidural anaesthesia is generally not recommended for use in type 2 or 3 VWD [67].…”

The use of desmopressin as a hemostatic agent: A concise review

“…von Willebrand disease DDAVP is most effective in patients with Type 1 VWD (see Table II for the classification of VWD), especially those who have normal VWF in storage sites (Type 1, platelet normal) [9,10]. In these patients FVIII, VWF, and bleeding time (BT) are usually corrected within 30 min and remain normal for 6-8 hr.…”

Principles of care for the diagnosis and treatment of von Willebrand disease

“…Several extensive reviews have recently been published on the pathophysiology, diagnosis and treatment of VWD to which the reader is referred. [3][4][5][6][7][8][9][10] The aim of the present review is to provide a concise practical outline of the diagnosis and treatment of VWD in Europe that could be useful for the general hematologist.…”