Treatment of Hemophilia C with FXI Inhibitors in Homozygous Type II Mutation
Clinical Scenario
Factor XI deficiency presenting with FXI antibodies (inhibitors) — a situation that arises with particular frequency in individuals who are homozygous for the type II mutation.
In this population, the formation of FXI antibodies can cause significant inhibition and create clinically important management challenges. Because inhibitor development is especially likely in those who are homozygous for the type II mutation, their presence substantially shapes the treatment approach.
Treatment Approach
Where inhibitors are present alongside residual factor XI activity, a plasma-based product approach may be applicable. The complete treatment algorithm — including sequencing, agent selection, and additional considerations — is available in the full protocol.
Dosages, full regimen, and clinical algorithm are in the structured protocol below.
References
Inhibitors are particularly likely to develop in people who are homozygous for the type II mutation.
FXI antibodies can cause significant inhibition and clinical problems.
Some inhibitors are associated with residual FXI activity and may be treated successfully with plasma products.