hematology and oncology
Von Willebrand Disease
Treatment of Von Willebrand Disease
Desmopressin von Willebrand factor(VWF) replacement when necessary Patients with von Willebrand disease (VWD) are treated only if they are actively bleeding or are undergoing an invasive procedure (eg, surgery, dental extraction). For patients with type 1 VWD,desmopressin, an analog ofvasopressin(antidiuretic hormone) that stimulates release of VWF into the plasma and may increase levels of factor VIII, can be helpful.Desmopressinis usually ineffective in type 2 and in type 3 VWD. In type 2B VWD,desmopressincan exacerbate thrombocytopenia. To ensure adequate response to the drug, physicians give patients a test dose and measure the response of VWF antigen.Desmopressin0.3 mcg/kg given in 50 mL of 0.9% saline solution IV over 15 to 30 minutes may enable patients to undergo minor procedures (eg, tooth extraction, minor surgery) without needing replacement therapy. If a replacement product is needed,desmopressinmay reduce the required dose. may be as effective as IV treatment and is often useful to prevent bleeding during minor surgical procedures. Hyponatremia . For patients with type 2 VWD, those with type 3 VWD, or patients with type 1 VWD who are undergoing more extensive invasive procedures, treatment involves replacement of VWF by infusion of intermediate-purity factor VIII concentrates, which contain components of VWF. These concentrates are virally inactivated and therefore do not transmit Human Immunodeficiency Virus (HIV) Infection or For women with heavy menstrual bleeding due to von Willebrand disease, a brief period of treatment withtranexamic acidby mouth or intranasaldesmopressinmay decrease bleeding.Tranexamic acidmay also be of use for patients with type 1 and type 2 VWD undergoing minor surgical procedures (eg, dental extraction, skin biopsy, excisional breast biopsy). 