The reproductive health of women with von Willebrand Disease unresponsive to DDAVP: results of an international survey. On behalf of the Subcommittee on von Willebrand Factor of the Scientific and Standardization Committee of the ISTH.
An international registry was established on the reproductive health of women with types of von Willebrand disease (vWd) unresponsive to DDAVP. Data was collected on 44 women from 16 treatment centers in nine countries. Severe menorrhagia requiring blood product therapy occurred at least once in 80% of the women for whom data was reported. Most of the reported episodes occurred prior to the diagnosis of vWd and/or the use of oral contraceptive (OC) therapy. OC therapy was clinically effective in the treatment of chronic menorrhagia in 22 of 25 (88%) women treated. Two of the women, however, were unable to tolerate chronic OC use and a third became refractory to treatment. Hysterectomy was performed in 10 of the 44 women (23%). The reported indication for six of the procedures was menorrhagia. Seventy percent were performed at two treatment centers, suggesting different thresholds for the performance of the procedure. There were 69 pregnancies reported in 31 of the 44 women. Fifteen of the pregnancies resulted in spontaneous abortions. The incidence of miscarriage was 22% and appeared clustered, with 10 of 15 of the miscarriages occurring in just four of the women.
Foster
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PA
Blood Research Institute, Blood Center of Southeastern Wisconsin, Milwaukee 53201-2178, USA.
D000022:Abortion, Spontaneous / Q000453:epidemiology / Q000209:etiology*; D000328:Adult; D001779:Blood Coagulation Factors / Q000627:therapeutic use; D001803:Blood Transfusion; D003278:Contraceptives, Oral, Hormonal / Q000627:therapeutic use; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use*; D005060:Europe / Q000453:epidemiology; D005260:Female; D006801:Humans; D007044:Hysterectomy; D008595:Menorrhagia / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology* / Q000628:therapy; D006473:Postpartum Hemorrhage / Q000453:epidemiology / Q000209:etiology; D011247:Pregnancy; D011256:Pregnancy Outcome; D012042:Registries; D017211:Treatment Failure; D014481:United States / Q000453:epidemiology; D014842:von Willebrand Diseases / Q000150:complications* / Q000188:drug therapy
D001779:Blood Coagulation Factors; D003278:Contraceptives, Oral, Hormonal; D003894:Deamino Arginine Vasopressin