Hemophilia A
ICD-10 D66 · ICD-11 3B10.0

Treatment of Severe and Moderately Severe Hemophilia A (FVIII <2 IU/dL) Without Inhibitors Undergoing a Major Invasive Procedure

Clinical Scenario

This protocol applies to patients with severe or moderately severe hemophilia A — defined by a factor VIII activity below 2 IU/dL — who do not have factor VIII inhibitors and are scheduled for or have undergone a major invasive procedure.

The absence of inhibitors is a critical qualifier. The perioperative setting introduces heightened bleeding risk that requires targeted haemostatic management throughout the procedural period.

Treatment Approach

Management centres on factor VIII concentrate replacement. The approach involves infusion of FVIII concentrates — either plasma-derived or standard half-life recombinant formulations — administered in relation to the invasive procedure.

Full regimen details, dosing strategy, and timing are available in the complete structured protocol below.
Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1016/j.jtha.2024.05.026

In individuals with severe and moderately severe hemophilia A without inhibitors undergoing a major invasive procedure, the ISTH Hemophilia Guideline Panel suggests either continuous or bolus infusion of plasma-derived or standard half-life recombinant FVIII concentrates (conditional recommendation, based on very low–certainty evidence ⨁◯◯◯).

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