Regulation and importance of factor VIII levels in hemophilia A carriers

Peter H. Cygan, Peter A. Kouides

Research output: Contribution to journalReview articlepeer-review

9 Scopus citations


Purpose of reviewTo summarize the recent literature related to female hemophilia A carriers with respect to prevalence in the population, the impact of baseline factor VIII levels and other influences on bleeding phenotype, and clinical management needs.Recent findingsMany female hemophilia A carriers are at risk for abnormal bleeding, yet they are underrecognized by healthcare providers and their bleeding symptoms are underreported. Low FVIII levels are consistently associated with clinically significant bleeding and correlate well with skewed X chromosome inactivation (XCI). Most interestingly, bleeding tendency is also observed in some hemophilia A carriers with normal factor VIII levels and requires further investigation. Well controlled studies investigating peripartum and periprocedural FVIII levels and adequate hemostatic treatment are necessary to inform management guidelines.SummaryPrevalence and bleeding tendency of hemophilia A carriers remain underreported, despite a significant proportion having low FVIII levels. Skewed XCI may explain low FVIII but does not explain the bleeding risk encountered in a larger proportion of hemophilia A carriers with random XCI and borderline/normal FVIII.

Original languageEnglish (US)
Pages (from-to)315-322
Number of pages8
JournalCurrent Opinion in Hematology
Issue number5
StatePublished - Sep 1 2021

All Science Journal Classification (ASJC) codes

  • Hematology


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