Testing for von Willebrand disease in women with menorrhagia: a systematic review.

OBJECTIVE

To review the evidence supporting screening of adult women with menorrhagia for von Willebrand disease.

DATA SOURCES

MEDLINE search from January 1,1990, to December 31, 2003, for articles in English, using keywords "menorrhagia," "von Willebrand disease," "diagnosis," and "screening," with a hand-search of bibliographies of identified articles, review of published abstracts, and discussion with experts.

METHODS OF STUDY SELECTION

One hundred seven articles meeting search criteria were reviewed. Articles included in the study were those that provided primary data on the prevalence of von Willebrand disease in adult women with menorrhagia, quality of life, surgical complications, and the effectiveness of medical therapy in women with menorrhagia and von Willebrand disease and test characteristics of screening tests for von Willebrand disease.

TABULATION, INTEGRATION, AND RESULTS: The reported prevalence of von Willebrand disease in women with menorrhagia ranged from 5-20% in 5 published studies. Comparison of results was limited by small sample sizes and large confidence intervals, as well as differences in the definitions of menorrhagia and von Willebrand disease used in the studies. Although menorrhagia in women with known von Willebrand disease has a substantial impact on quality of life, there are no data suggesting that this impact is substantially greater than that of menorrhagia in women without von Willebrand disease. Data on the risk of surgical bleeding in women with von Willebrand disease are limited, with only 3 studies with a total of 29 patients identified. Data on the effectiveness of specific therapies are also limited; only one controlled trial was identified. Of single tests for screening, one study of the ristocetin cofactor assay had a sensitivity of 79% and specificity of 90%. Studies of a test of platelet adhesion and aggregation resulted in pooled sensitivities of 83-94% and specificities of 80-88%; however, significant heterogeneity was present.

CONCLUSION

There are inadequate data to justify routine testing for von Willebrand disease in adult women with menorrhagia outside of the research setting.