Table 3.

Mean steady-state FIX levels and prophylaxis status by participant

ParticipantPre-AMT-060 gene transferPost-AMT-060 gene transfer
FIX activity at diagnosis, IU/dLHemophilia B phenotypeFIX prophylaxisPresumed CRM status* (activity/antigen ratio)Mean steady-state FIX, IU/dL activity (95% CI)FIX prophylaxisHemophilia B phenotypeLessening of severity
<1 Severe Yes +(0.1) 6.2 (5.8-6.6) No Mild Yes 
<1 Severe Yes +(0.4) 4.7 (4.5-5.0) No Moderate Yes 
<1 Severe Yes (NC) 1.3 (–0.7 to 3.2) Yes Moderate Yes 
1.5 Moderate Yes (1.4) 6.8 (6.3-7.3) No Mild Yes 
<1 Severe Yes (1.2) 3.0 (2.6-3.4) No Moderate Yes 
<1 Severe Yes (1.1) 12.7 (11.9-13.5) No Mild Yes 
<1 Severe Yes +/−(0.7) 6.4 (6.0-6.7) No Mild Yes 
<1 Severe No (1.6) 6.8 (5.8-7.7) No Mild Yes 
<1 Severe Yes +(0.0) 3.1 (2.8-3.3) No Moderate Yes 
10 <1 Severe Yes (1.4) 5.8 (5.4-6.2) No Mild Yes 
ParticipantPre-AMT-060 gene transferPost-AMT-060 gene transfer
FIX activity at diagnosis, IU/dLHemophilia B phenotypeFIX prophylaxisPresumed CRM status* (activity/antigen ratio)Mean steady-state FIX, IU/dL activity (95% CI)FIX prophylaxisHemophilia B phenotypeLessening of severity
<1 Severe Yes +(0.1) 6.2 (5.8-6.6) No Mild Yes 
<1 Severe Yes +(0.4) 4.7 (4.5-5.0) No Moderate Yes 
<1 Severe Yes (NC) 1.3 (–0.7 to 3.2) Yes Moderate Yes 
1.5 Moderate Yes (1.4) 6.8 (6.3-7.3) No Mild Yes 
<1 Severe Yes (1.2) 3.0 (2.6-3.4) No Moderate Yes 
<1 Severe Yes (1.1) 12.7 (11.9-13.5) No Mild Yes 
<1 Severe Yes +/−(0.7) 6.4 (6.0-6.7) No Mild Yes 
<1 Severe No (1.6) 6.8 (5.8-7.7) No Mild Yes 
<1 Severe Yes +(0.0) 3.1 (2.8-3.3) No Moderate Yes 
10 <1 Severe Yes (1.4) 5.8 (5.4-6.2) No Mild Yes 

Only values at least 10 d after the preceding FIX administration are included.

CRM, cross-reactive material.

*

Baseline measurements for CRM were not collected.

The majority of individuals showed post-AMT-060 activity/antigen ratios of ∼1:1. Ratios lower than 1:1 are suggestive of CRM positivity.

Used on-demand FIX replacement before study entry.

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