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. 2020 Nov 24;4(22):5785–5796. doi: 10.1182/bloodadvances.2020002731

Table 2.

Patient characteristics stratified in 4 subgroups with distinct signatures of FVIII-binding antibodies developed during the HIPS study

Patient no. F8 mutation Age at first FVIII infusion, mo Peak FVIII inhibitor titer, BU/mL First FVIII inhibitor detection Ethnic background Family history of hemophilia A Family history of FVIII inhibitors
Subgroup 1: no FVIII-binding IgG antibodies
 1 Missense 9.1 <0.4 NA White No No
 2 Intron 22 inversion 12.7 <0.4 NA White Yes Yes
 3 Missense 2.8 <0.4 NA White Yes No
 4 Missense 2.5 <0.4 NA Black Yes No
 5 Duplication 7.8 <0.4 NA White No No
 6 Frameshift 16.6 <0.4 NA White Yes Yes
 7 Nonsense 6.7 <0.4 NA White Yes Unknown
Subgroup 2: non-neutralizing FVIII-binding antibodies
 8 Intron 22 inversion 9.3 <0.4 NA White No No
 9 Missense 19.9 <0.4 NA Black Yes Yes
 10 Intron 22 inversion 3.6 <0.4 NA White No No
 11 Missense 13.5 <0.4 NA White No No
 12 Intron 1 inversion 12.0 <0.4 NA White No No
 13 Missense 10.7 <0.4 NA White No No
 14 Intron 22 inversion 0.4 <0.4 NA White Yes Yes
Subgroup 3: transient FVIII inhibitors
 15 Frameshift 14.7 1.8 ED20 Black Yes Yes
 16 Intron 22 inversion 9.7 1.4 ED12 White Yes No
Subgroup 4: persistent FVIII inhibitors
 17 Large deletion 10.2 4.5 ED20 Hispanic No No
 18 Large deletion 12.0 7 ED6 Hispanic Yes Unknown
 19 Intron 22 inversion 12.6 796 ED10 White Yes Unknown
 20 Duplication 12.0 9.5 ED10 Hispanic Yes Yes
 21 Intron 22 inversion 10.3 323 ED10 White Yes Unknown
 22 Intron 22 inversion 6.3 3049 ED10 Asian or Pacific Yes No
 23 Intron 22 inversion 9.2 124 ED11 White Yes Yes

NA, not applicable.