Background:

The World Federation of Hemophilia (WFH) has developed the third edition of the WFH Guidelines for the Management of Hemophilia.

Methods:

This 2020 version of the WFH treatment guidelines incorporates evidence-based and Trustworthy Consensus-Based Statement (TCBS) approaches in conformance with established international standards for clinical practice guidelines.The TCBS process produces evidence-informed recommendationsthrough a transparent process that incorporates both the available evidence, identified in a systematic approach to reduce biases, and expert clinical advice.

Results:

Guided by content leads (AS, GP), a guidelines methodology expert (SZL), and 11 chapter leads, the original 7 chapters of the 2012 version of the guidelines were updated and 4 new chapters were added (Table 1).Each chapter was assigned an expert panel composed of up to 10 members, healthcare professionals with clinical expertise, and patients/caregivers, with the latter making up 25% of each chapter panel. A total of 50 panelists were assigned to the 11 chapters, with some panelists serving on more than one panel.

Each chapter topic went through a systematic review of the published literature, and each study was screened for eligibility based on apriori defined criteria on population, interventions, comparisons, and outcomes.Data was extracted from included studies and evidence tables developed.Evidence-based recommendations were drafted by the chapter panel and each set of recommendations went through a Delphi consensus process.The threshold for achieving consensus was 80% agreement among panel voters.

Across all chapters, 344 recommendationsachieved consensus. Recommendations not achieving consensus were included as discussion points in the final manuscripts.

Chapter title
Principles of Care
General and Comprehensive Care of Hemophilia
Laboratory Diagnosis and Monitoring
Genetic Assessment
Hemostatic Agents
Prophylaxis: Regular Factor Replacement Therapy
Treatment of Specific Hemorrhages
Inhibitors to Clotting Factor
Specific Management Issues
MSK Complications
Outcome Assessment

Conclusions:

This third edition of the WFH Guidelines for the Management of Hemophilia are trustworthy, reliable, evidence-informed, and expert-driven recommendations that will guide the treatment and management of hemophilia around the world.