Prevalence of symptoms in hemophilia carriers in comparison with the general population: a systematic review

INTRODUCTION: Hemophilia is well known in males, but poorly recognized in hemophilia carriers, who may have a hemorrhagic tendency, and the symptoms may be frequent and severe. Few studies have been done evidencing this bleeding in female carriers of the hemophilia gene. OBJECTIVES: To verify the prevalence of hemorrhagic symptoms in HC, compared to women in the general population. MATERIAL AND METHOD: The articles published between October 1996 and November 2016 were searched in the PubMed, Scielo, Lilacs, Web of Science, Scopus and Cochrane Central databases. RESULTS AND DISCUSSION: Seventy-five articles were found in electronic databases and 2 additional articles, through manual search in journal summaries and bibliographical references of other review articles. There is a limitation as to the number of studies that explore the association between the risk of hemorrhagic events and HC A or B. Among the few existing studies, there is a methodological difference, evidenced by control groups with distinct recruitments, divergent questionnaires and non-standardized concepts. CONCLUSION: This review verified the existence of a higher prevalence of hemorrhagic symptoms in the HC in some outcomes, however, due to the limitations of the few studies found, there is still insufficient evidence to state that the HC has a greater hemorrhagic tendency in relation to the general population.