Hemostatic agents

The different hemostatic agents used for treatment of bleeding and/or handling different clinical procedures in VWD patients include the following drugs:

Choice of treatment

The choice of treatment depends on several factors:

  • Nature of the bleed or invasive procedure.

  • Subtype and severity of VWD - level of functional VWF and FVIII.

  • Previous bleeding history and response to treatment.

  • Duration of treatment - single doses or a long-term treatment.

  • Outcome of the DDAVP test - post DDAVP level and half-life of functional VWF and FVIII.Age of the patient. Restricted use of DDAVP is advised in frail elderly and the youngest children of less than 2 years of age - due to increased risk symptomatic hyponatremia in and an increased risk of thrombotic complications in patients with cardiovascular risk factors, including high age.

  • The presence of other diseases that may contraindicate use of a therapeutic agent.

  • Pregnancy and delivery.

Suggested treatment options are given in Figure 1, in the text below and in appendix 1.

Figure 1: Management algorithm. Tranexamic acid should be given to all patients unless there is a contraindication. Repetitive doses of tranexamic acid will load the tissue and are often useful for hemostasis by inhibiting early fibrinolysis. A single dose of tranexamic acid can be used in case there is a high risk of thrombosis during an acute bleed or major surgery. DDAVP is generally inefficient or contraindicated in patients with type 1C VWD in the setting of surgery, and in type 2B and type 3 VWD patients. Desmopressin may be used in some instances of mild bleeding for type 2 VWD, mainly some 2M variants with confirmed responses in the DDAVP trial. The choice between VWF replacement therapies (FVIII/VWF 1:1, 1:2, 0:1) is made based on local practices and the clinical assessment of thrombosis risk. OD: on demand; DDAVP: desamino-8-arginine vasopressin.