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Guidelines adherence reduces bleeding risk after surgery and childbirth for VWD patients

Presented by
Dr Brian Craven, Royal Free London NHS Foundation Trust, United Kingdom
ISTH 2020

A retrospective, single-centre cohort study following 30 patients with Von Willebrand Disease (VWD) who underwent major surgery or childbirth revealed that adherence to current guidelines can reduce the risk of major bleeding events to an acceptable range [1].

Patients with VWD, the most common heritable bleeding disorder, are at increased bleeding risk from invasive procedures. To prevent complications, prophylactic treatment is often administered and recommended in current guidelines. For patients undergoing major surgery, several factor replacement options exist. The current study assessed how closely real-world practice in large centres is aligned with national guidelines and how the recommendations influence post-operative bleeding and/or thrombosis.

The analysis included 30 VWD patients who underwent major surgery or childbirth (both vaginal delivery or caesarean section) and received factor replacement. Patients were eligible to receive any available factor replacement concentrate. The timing of dosing of the factor concentrates relative to the previous dose was collected, as well as the duration of factor replacement. Surgical complications (i.e. bleeding or thrombotic complications) were recorded.

Over a 13-year period, 30 participants underwent 41 major operations or childbirths. The majority of the patients undergoing major surgery achieved 100 IU/dL at the time of surgery, the level recommended by the UK guideline [2]; all patients maintained levels over 50 IU/dL for the first 7 days following the surgery, but not all patients achieved target Von Willebrand factor ristocetin cofactor (VWF:RCo) level consistently for 7 days following the surgery.

The patients undergoing childbirth received the first dose of factor replacement at the beginning of labour. Of the patients who gave birth, 98% were at or above target levels for factor VIII and VWF:RCo. No patient with type 3 VWD underwent childbirth during the study period.

Only 2 patients developed bleeding complications, 1 of them major with a bleeding rate of 2.4%. Over the study period, no episode of thrombosis was observed, which was unexpected because many patients achieved supra-physiologically factor VIII levels particularly in the first 5 days post-procedure.

The authors concluded that by close adherence to national guidelines for factor VIII and VWF:RCo replacement in VWD patients undergoing major surgery or delivery, the risk of major bleeding can be reduced to an acceptable level of 2.4%. However, even within a tertiary referral centre, adherence to guidelines showed to be suboptimal. In the future, levels between certain values should be maintained (not just above a certain level) to try to minimise the risk of thrombosis. Continuous dosing, greater knowledge of the pharmacokinetics of the factor replacements between different VWD patients, and the advent of new therapeutic options, e.g. recombinant VWF, would be suitable to reach this goal.

  1. Craven B et al. PB1591, ISTH 2020 Virtual Congress, 12-14 July.
  2. Laffan MA et al. Br J Haematol 2014;167:453-465.


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