Guideline Video

Guideline Resources

  • Management of Women with Type 2B von Willebrand Disease During Pregnancy and Postpartum
  • International Society on Thrombosis and Haemostasis
  • June 4, 2026
  • Summary
  • Full-text

Video Transcription

Just published June 4th, 2026, the International Society on Thrombosis and Haemostasis’ newest consensus statement on Management of Women with Type 2B von Willebrand Disease During Pregnancy and Postpartum.

The purpose of this consensus statement is to standardize care, guide management, and improve clinical outcomes for women with type 2B von Willebrand disease, or VWD, during pregnancy and postpartum. 

In today’s rapid update, we’ll just be going over a summary of the key guidance statements so for the full consensus statement, make sure to check it out on guidelinecentral.com

Let’s get started. 

Starting with the section on Diagnosis and Prenatal Counseling

  • In girls and women with abnormal initial von Willebrand factor, or VWF, tests suggestive of type 2B VWD, the consensus advises that the diagnosis to be established ideally before becoming pregnant. 
  • The consensus suggests that type 2B VWD should be considered as a differential diagnosis in all pregnant women with unexplained and progressive thrombocytopenia. 
  • In women presenting during pregnancy with suspected type 2B VWD, the consensus advises that the threshold to perform genetic testing may be lower than in the non-pregnant state to minimize misdiagnosis due to pregnancy-induced changes of hemostatic parameters. 
  • The consensus advises appropriate prenatal counselling by a multidisciplinary team (MDT) expert in VWD. 
  • The consensus suggests that in women with type 2B VWD, hemoglobin and ferritin levels be checked regularly before and during pregnancy. Iron substitution must be introduced - when indicated- to avoid iron deficiency and/or anemia during pregnancy. 
  • The consensus suggests that all women with type 2B VWD are a priori at high risk of bleeding during pregnancy and postpartum, irrespective of previous bleeding history. 

Next the section on Monitoring and Management During Pregnancy

  • When assessing bleeding risk, the consensus suggests that laboratory test results should not to be evaluated individually. Combined low VWF levels & thrombocytopenia represents a higher risk than each alone. Thrombocytopathy, reported in association with some 2BVWD variants adds to the risk. 
  • The consensus suggests that plasma VWF and FVIII levels and platelet count should be monitored at the beginning of the pregnancy, at least once during the third trimester, close to delivery, and at least once in the first week after delivery. The frequency and timing of testing may be adapted as per clinical judgement. 
  • Due to the complexity of type 2B VWD, and the high risk of pregnancy-related bleeding events, the consensus advises that labor and delivery should be managed by a MDT, in a specialized center with access to factor concentrates and blood products, including platelets. It is important to ensure that haemostasias advice and appropriate laboratory monitoring is readily available to the obstetric team. 
  • The consensus suggests that in women with type 2B VWD who experienced bleeding requiring VWF replacement therapy during pregnancy, subsequent prophylactic administration of VWF concentrate may be considered. The dosing, intervals of administration, and duration of prophylaxis are determined based on bleeding risk, clinical response, and VWF/FVIII levels.

And last the section on Management of Delivery and Postpartum Care

  • The consensus advises that a delivery plan should be formulated in the third trimester using a shared decision-making process with the patient and MTD members. 
  • The consensus suggests that the mode of delivery mostly depends on obstetric indications. Delivery must be achieved with the least traumatic method to the baby, avoiding fetal blood sampling, fetal scalp monitoring, ventouse delivery, and mid-cavity forceps. The consensus also advises avoidance of external cephalic version during pregnancy for breech presentation. 
  • The consensus suggests that VWF-containing concentrate and blood products should be readily available during pregnancy, at the time of delivery, and postpartum for all women with 2B VWD. 
  • The safe level of VWF and platelet count for neuraxial anesthesia in women with type 2B VWD is not known, and the consensus suggests that the risk must be assessed individually. Intervention must be avoided if the VWF activity level is <50 IU/dL and/or platelet count <70x10^9/L. The trough levels of VWF activity during neuraxial catheter stay must be >50 IU/dL. The removal of the catheter needs approximately the same levels of VWF and platelet count as for its placement. 
  • The consensus advises that desmopressin to be avoided in women with type 2B VWD during pregnancy and postpartum due to the risk of causing/worsening thrombocytopenia. 
  • The consensus suggests that both laboratory results and clinical scenarios must guide replacement therapy with VWF, FVIII, and platelets for delivery and postpartum. 
  • The consensus suggests that the targeted VWF activity and FVIII levels for delivery are ≥ 100 IU/dL. Trough VWF/FVIII level should be kept at ≥ 80 IU/dL during the first 3-5 days after vaginal delivery and during 5-7 days after C-section. Replacement therapy could be continued beyond this period if excessive bleeding or a significant risk of bleeding persists with an adjustment of treatment according to clinical circumstances. 
  • The consensus suggests that platelet transfusion be administered when the platelet count is <30x10^9/L at the start of vaginal delivery or <50x10^9/L in case of cesarean delivery. Repeated platelet transfusions may be needed to achieve counts >30x10^9/L during the first 3-5 days after delivery. 
  • If there is excessive bleeding during delivery or postpartum despite correct VWF replacement, the consensus suggests that platelet transfusion be administered to achieve a platelet count >50x10^9/L. 
  • The consensus suggests that blood count, plasma VWF activity and FVIII levels should be checked at least once in the first 24h after delivery in all women with type 2B VWD. In women receiving VWF and/or platelet replacement therapy, levels must be regularly monitored during the entire treatment period. 
  • The consensus suggests that women with type 2B VWD undergo individualized venous thromboembolism risk assessment. If pharmacologic thromboprophylaxis is indicated, it should be administered for the minimum necessary duration, with close clinical surveillance and appropriate laboratory monitoring of haemostatic parameters. 
  • The consensus advises the use of postpartum tranexamic acid according to the ASH ISTH NHF WFH 2021 guidelines which favor administration of tranexamic acid over not. Tranexamic acid may be given via the oral or IV route. The recommended oral dose is ~ 25mg/kg 3 times per day for 10 to 14 days or longer if blood loss remains heavy. Women who intend to breastfeed should be provided education about benefits and the safety of tranexamic acid during breastfeeding. 
  • In cases of excessive postpartum bleeding, the consensus suggests immediate, concurrent haemostatic and obstetric assessment. Haemostatic therapy should be guided by bleeding phenotype and laboratory results, while obstetric evaluation should identify and manage obstetric causes of haemorrhage. 
  • After discharge, the consensus advises that the team should keep close contact with the patient until the resolution of lochia. In case of significant bleeding, the patient will need an immediate assessment at the obstetric and haemostasias centers undergoing to rule out / manage obstetric causes for bleeding. 
  • If prenatal testing is not done, the consensus suggests that screening for type 2B VWD in the neonate may be done using cord blood sampling. Until the diagnosis of type 2B VWD is excluded, vaccines must be given subcutaneously with the smallest gauge needle. Vitamin K should be given orally if there is the delay in the diagnosis or the diagnosis of type 2B VWD is confirmed. The drug may also be administered subcutaneously if appropriate formulation is available and approved.

And there you have it. Make sure to check out the full consensus statement from the International Society on Thrombosis and Haemostasis and other related clinical decision support tools at guidelinecentral.com.

Sign up for alerts and stay informed on the latest published articles and guidelines.