Estrogens and surgery in women with von Willebrand's disease

Abstract

While receiving estrogen therapy, three women with classic type I von Willebrand's disease showed significantly less bleeding and improved hemostasis. One took estrogens to prevent menopausal symptoms, and two used oral contraceptives. These women underwent major surgery without excessive bleeding and without requiring cryoprecipitate or other blood components. Prior to estrogen therapy, each woman had received cryoprecipitate or fresh frozen plasma to stop bleeding from dental extractions or other surgical procedures. Furthermore, two other women with type I von Willebrand's disease also exhibited improved hemostasis while taking oral contraceptives. These experiences suggest a short course of estrogen therapy may effectively prepare some women with von Willebrand's disease for elective surgery.

PIP: Von Willebrand's disease causes spontaneous bleeding from mucocutaneous surfaces and excessive blood loss after surgery. Some women with the disease have less spontaneous hemorrhage and show improved coagulation while taking oral contraceptives (OCs) or while pregnant; estrogens are believed responsible for these improvements. 3 women are described who had previously received infusions of either fresh frozen plasma or cryoprecipitate to stop postoperative hemorrhage, and exhibited prolonged bleeding times, abnormal platelet retention results, and decreased plasma levels of factor VIII-related coagulant, factor VIII-related antigen, and factor VIII-related von Willebrand factor. However while taking estrogens they experienced cessation of spontaneous bleeding episodes and exhibited normal coagulation test results; all observed less epistaxis and spontaneous bruising during pregnancy. Continuing estrogen therapy, each woman underwent major surgery with no abnormal bleeding an no blood component therapy. 2 other women with type 1 von Willebrand's disease also exhibited improved hemostasis while taking OCs. It is unclear how estrogens stimulate synthesis of the various factor VIII components and for certain women with von Willebrand's disease certain estrogens may improve hemostasis more effectively than others. Estrogens might obviate the need for cryoprecipitate or fresh frozen plasma to assure hemostasis, thereby eliminating the threat of transfusion-induced hepatitis and reducing surgical costs.