Prevalence and Incidence of Non-neutralizing Antibodies in Congenital Hemophilia A- A Systematic Review and Meta-Analysis.

摘要: Objectives: In hemophilia A the presence of non-neutralizing antibodies (NNAs) against Factor VIII (FVIII) may predict development neutralizing (inhibitors) and accelerate clearance administrated FVIII concentrates. This systematic review aimed to assess: (1) prevalence incidence NNAs in patients with congenital without inhibitors (2) association between patient treatment characteristics. Methods: We conducted a search MEDLINE, Embase, Web Science Cochrane database. included cross-sectional longitudinal studies reporting on B, who were inhibitor-negative at start observation period. Data extracted on: type severity, characteristics, NNA incidence, assays inhibitor development. Two independent reviewers performed study selection, data extraction risk bias assessment, using adapted criteria Joanna Briggs Institute. Studies classified as high-quality when ≥5/9 met. both quality met: use positive controls competition establish FVIII-specificity. reported for each study. The pooled was assessed well-designed previously treated patients, employing assays. Results: from 2,723 A, derived 28 studies. Most (19/28) none B. Study design high 16/28 assay 9/28 Various used, predominantly ELISA (18/28) different cut-off values. found large variety (Range, 0-100%). 25% (95% CI, 16-38%). new one (0.01 per person-exposure day). Conclusion: identified that heterogeneous design, population type, ranging 0 100% A. including only performing