Haemostatic therapy includes antifibrinolytic agents (tranexamic acid and aminocaproic acid) and/or DDAVP or desmopressin (1-desamino-8-d-arginine vasopressin),

a synthetic vasopressin that stimulates the release of von Willebrand factor (VWF) from endothelial cells, in addition to replacement treatment. Case-control studies also report that women with VWD have significantly higher rates of heavy bleeding that ended the pregnancy compared with controls. Two different series of women with VWD reported a lower prevalence of primary postpartum haemorrhage (PPH) and a higher prevalence of the secondary PPH compared with haemophilia carriers. The most recent data documenting and comparing the incidence of PPH in women with VWD and controls come from a US discharge database, reporting BVD-523 in vitro that 6% of pregnancies in such women were complicated by PPH compared to 4% of controls [9]. Peripartum management of women with Selleck Barasertib VWD at the beginning requires a laboratory evaluation for VWD that includes a basic coagulation panel, VWF antigen (VWF:Ag)

assay, VWF ristocetin cofactor (VWF:RCo) assay and FVIII levels. Treatment should be instituted if the levels of VWF:RCo and FVIII are <50 IU dL−1 before any invasive procedure and delivery. The mainstays of therapy are desmopressin (DDAVP) and plasma concentrates that contain VWF. DDAVP may be used in women with type 上海皓元医药股份有限公司 1 VWD; recent data indicate that some individuals have accelerated clearance of VWF; therefore, even patients with type 1 may benefit from a test dose of DDAVP and subsequent measurement of VWF:RCo to document treatment efficacy [10]. In women with type 2, the main problem is that, despite an increase in secretion of VWF after treatment with DDAVP, the VWF secreted retains its intrinsic molecular dysfunction. As a result, the use of VWF concentrates is the preferred

therapy for type 2 VWD [11]. However, a small subset of women with type 2 VWD respond to DDAVP and identification of those individuals requires a test dose of DDAVP and subsequent measurement of VWF:RCo 1 and 4 h after infusion. If the VWF:RCo corrects postdose, DDAVP is an acceptable treatment for this subset of women. Flushing, headache, GI complaints and transient hypo or hypertension are minor adverse effects of DDAVP. Repeated dosing is discouraged as it may lead to water retention and hyponatremia. DDAVP is safe for the foetus because it does not cross the placenta in detectable amounts [11]. According to previous reports, women with type 3 VWD lack the physiological rise in VWF during pregnancy. Only a few reports exist regarding the management of pregnancy and delivery in women with VWD type 3, hence few data about the clinical problems and their appropriate management are available. These patients could particularly be considered for prophylactic treatment with DDAVP.