Factor VIII and Factor IX

Table 2. HTC Population Profile Patient Characteristics, Factor VIII and Factor IX Deficiencies, Data Reported From 1/1/2012 through 3/31/2021

HTC Population Profile Patient Characteristics, Factor VIII and Factor IX
Hemophilia A (Factor VIII deficiency)(n=21400) Hemophilia B (Factor IX deficiency)(n=6864)
Factor level >=40% Mild Moderate Severe Severity
Unknown
Factor level >=40% Mild Moderate Severe Severity
Unknown
# (%) # (%) # (%) # (%) # (%) # (%) # (%) # (%) # (%) # (%)
# of patients 1435 (100) 6877 (100) 3455 (100) 9224 (100) 409 (100) 568 (100) 2115 (100) 2383 (100) 1690 (100) 108 (100)
Age (years) <2 24 (2) 164 (2) 86 (2) 228 (2) 24 (6) 13 (2) 70 (3) 77 (3) 58 (3) * *
2-10 189 (13) 1029 (15) 615 (18) 1718 (19) 64 (16) 106 (19) 315 (15) 450 (19) 320 (19) 19 (18)
11-19 311 (22) 1556 (23) 728 (21) 1964 (21) 57 (14) 107 (19) 459 (22) 504 (21) 301 (18) 16 (15)
20-44 574 (40) 2252 (33) 1317 (38) 4112 (45) 169 (41) 244 (43) 663 (31) 766 (32) 677 (40) 35 (32)
45-64 223 (16) 1155 (17) 483 (14) 971 (11) 67 (16) 66 (12) 386 (18) 377 (16) 257 (15) 21 (19)
65+ 114 (8) 721 (10) 226 (7) 231 (3) 28 (7) 32 (6) 222 (10) 209 (9) 77 (5) * *
Sex Male 245 (17) 5752 (84) 3393 (98) 9180 (100) 162 (40) 89 (16) 1607 (76) 2361 (99) 1683 (100) 57 (53)
Female 1190 (83) 1125 (16) 62 (2) 44 (0) 247 (60) 479 (84) 508 (24) 22 (1) 7 (0) 51 (47)
Ethnicity Hispanic, Latino/a, or Spanish origin 215 (15) 1390 (20) 729 (21) 1614 (17) 82 (20) 34 (6) 164 (8) 150 (6) 318 (19) 10 (9)
Not Hispanic, Latino/a, or Spanish origin 1162 (81) 5361 (78) 2680 (78) 7511 (81) 285 (70) 507 (89) 1910 (90) 2213 (93) 1349 (80) 85 (79)
Unknown 58 (4) 126 (2) 46 (1) 99 (1) 42 (10) 27 (5) 41 (2) 20 (1) 23 (1) 13 (12)
Race American Indian/Alaska Native 39 (3) 88 (1) 46 (1) 76 (1) 7 (2) * * * * 28 (1) 17 (1) * *
Asian 30 (2) 200 (3) 128 (4) 480 (5) 18 (4) * * 33 (2) 34 (1) 85 (5) * *
Black or African American 102 (7) 379 (6) 467 (14) 1430 (16) 39 (10) 30 (5) 163 (8) 99 (4) 251 (15) * *
Native Hawaiian or other Pacific Islander * * 8 (0) 12 (0) 53 (1) * * * * * * 10 (0) 8 (0) * *
White 1170 (82) 5837 (85) 2662 (77) 6787 (74) 284 (69) 497 (88) 1847 (87) 2165 (91) 1274 (75) 95 (88)
More than one of these * * 98 (1) 38 (1) 148 (2) * * * * 10 (0) 15 (1) 14 (1) * *
Unknown 79 (6) 267 (4) 102 (3) 250 (3) 55 (13) 27 (5) 54 (3) 32 (1) 41 (2) 7 (6)
Insurance Status Insured 1368 (95) 6586 (96) 3277 (95) 8819 (96) 350 (86) 423 (74) 1857 (88) 1840 (77) 1619 (96) 77 (71)
Uninsured 36 (3) 184 (3) 111 (3) 214 (2) 29 (7) 112 (20) 223 (11) 511 (21) 42 (2) 18 (17)
Unknown 31 (2) 107 (2) 67 (2) 191 (2) 30 (7) 33 (6) 35 (2) 32 (1) 29 (2) 13 (12)
History of HCV infection Yes 46 (3) 874 (13) 714 (21) 2283 (25) 30 (7) 6 (1) 204 (10) 393 (16) 517 (31) 10 (9)
No 1083 (75) 5239 (76) 2524 (73) 6467 (70) 233 (57) 392 (69) 1611 (76) 1710 (72) 1074 (64) 57 (53)
Unknown 306 (21) 764 (11) 217 (6) 474 (5) 146 (36) 170 (30) 300 (14) 280 (12) 99 (6) 41 (38)
History of HIV infection Yes 11 (1) 107 (2) 186 (5) 934 (10) 12 (3) * * 28 (1) 45 (2) 131 (8) * *
No 1123 (78) 5933 (86) 3024 (88) 7739 (84) 251 (61) 398 (70) 1768 (84) 2023 (85) 1439 (85) 62 (57)
Unknown 301 (21) 837 (12) 245 (7) 551 (6) 146 (36) * * 319 (15) 315 (13) 120 (7) * *

Note. Hemophilia severity is defined based on the level of factor activity circulating in the blood. In this table, “Factor level >=40%” is defined as greater than or equal to 40% baseline clotting factor activity; “mild” is defined as greater than 5% and less than 40% baseline clotting factor activity; “moderate” is defined as 1–5% baseline clotting factor activity; “severe” is defined as less than 1% baseline clotting factor activity; and “severity unknown” is reported if the factor activity is missing or unknown. Males and females with factor VIII and IX deficiency are classified the same.

The HTC Population Profile contains 51 transsexual individuals. For confidentiality purposes, the number of transsexual patients is too small to report by year or other characteristics. Transsexual patients have been included in the counts of male and female according to the sex assigned to them at birth since hemophilia and von Willebrand disease, the most common congenital bleeding disorders, affect the sexes differently. On August 10, 2020, the responses for this question changed such that “Male to Female” (i.e., transsexual) and “Female to Male” were removed and “Intersex” was added. The number of intersex patients is too small to report by year or other characteristics; therefore, these counts have been suppressed to protect patient confidentiality.

* Counts of five or fewer have been suppressed to protect patient confidentiality. Additional cells may be suppressed to prevent derivation of these counts by subtraction.