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 3/31/2019
| Hemophilia A (Factor VIII deficiency) (n=19192) | Hemophilia B (Factor IX deficiency) (n=6095) | ||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Mild | Moderate | Severe | Severity Unknown | Mild | Moderate | Severe | Severity Unknown | ||||||||||
| # | (%) | # | (%) | # | (%) | # | (%) | # | (%) | # | (%) | # | (%) | # | (%) | ||
| # of patients | 7095 | (100) | 3148 | (100) | 8615 | (100) | 334 | (100) | 2276 | (100) | 2168 | (100) | 1552 | (100) | 99 | (100) | |
| Age (years) | <2 | 153 | (2) | 93 | (3) | 218 | (3) | 16 | (5) | 65 | (3) | 54 | (2) | 53 | (3) | * | * |
| 2-10 | 1130 | (16) | 590 | (19) | 1710 | (20) | 53 | (16) | 390 | (17) | 409 | (19) | 301 | (19) | 19 | (19) | |
| 11-19 | 1571 | (22) | 699 | (22) | 1922 | (22) | 36 | (11) | 482 | (21) | 471 | (22) | 290 | (19) | 15 | (15) | |
| 20-44 | 2365 | (33) | 1149 | (36) | 3682 | (43) | 153 | (46) | 743 | (33) | 719 | (33) | 621 | (40) | 30 | (30) | |
| 45-64 | 1210 | (17) | 430 | (14) | 894 | (10) | 56 | (17) | 386 | (17) | 343 | (16) | 224 | (14) | 25 | (25) | |
| 65+ | 666 | (9) | 187 | (6) | 189 | (2) | 20 | (6) | 210 | (9) | 172 | (8) | 63 | (4) | * | * | |
| Sex† | Male | 5280 | (74) | 3091 | (98) | 8576 | (100) | 140 | (42) | 1506 | (66) | 2148 | (99) | 1546 | (100) | 61 | (62) |
| Female | 1815 | (26) | 57 | (2) | 39 | (0) | 194 | (58) | 770 | (34) | 20 | (1) | 6 | (0) | 38 | (38) | |
| Ethnicity | Hispanic, Latino/a, or Spanish origin | 1343 | (19) | 645 | (20) | 1468 | (17) | 63 | (19) | 162 | (7) | 135 | (6) | 289 | (19) | 11 | (11) |
| Not Hispanic, Latino/a, or Spanish origin | 5630 | (79) | 2472 | (79) | 7077 | (82) | 253 | (76) | 2070 | (91) | 2015 | (93) | 1247 | (80) | 82 | (83) | |
| Unknown | 122 | (2) | 31 | (1) | 70 | (1) | 18 | (5) | 44 | (2) | 18 | (1) | 16 | (1) | 6 | (6) | |
| Race | American Indian/Alaska Native | 108 | (2) | 40 | (1) | 68 | (1) | * | * | * | * | 27 | (1) | 11 | (1) | * | * |
| Asian | 182 | (3) | 117 | (4) | 434 | (5) | 18 | (5) | 35 | (2) | 30 | (1) | 80 | (5) | * | * | |
| Black or African American | 423 | (6) | 427 | (14) | 1317 | (15) | 33 | (10) | 171 | (8) | 88 | (4) | 231 | (15) | 6 | (6) | |
| Native Hawaiian or other Pacific Islander | 8 | (0) | 10 | (0) | 53 | (1) | * | * | * | * | 12 | (1) | 10 | (1) | * | * | |
| White | 6047 | (85) | 2425 | (77) | 6425 | (75) | 245 | (73) | 2008 | (88) | 1978 | (91) | 1172 | (76) | 88 | (89) | |
| More than one of these | 85 | (1) | 42 | (1) | 130 | (2) | * | * | 10 | (0) | 9 | (0) | 15 | (1) | * | * | |
| Unknown | 242 | (3) | 87 | (3) | 188 | (2) | 28 | (8) | 47 | (2) | 24 | (1) | 33 | (2) | * | * | |
| Insurance Status | Insured | 6796 | (96) | 3030 | (96) | 8320 | (97) | 300 | (90) | 1931 | (85) | 1719 | (79) | 1507 | (97) | 79 | (80) |
| Uninsured | 217 | (3) | 88 | (3) | 214 | (2) | 22 | (7) | 291 | (13) | 432 | (20) | 37 | (2) | 12 | (12) | |
| Unknown | 82 | (1) | 30 | (1) | 81 | (1) | 12 | (4) | 54 | (2) | 17 | (1) | 8 | (1) | 8 | (8) | |
| History of HCV infection | Yes | 844 | (12) | 660 | (21) | 2204 | (26) | 31 | (9) | 189 | (8) | 379 | (17) | 491 | (32) | 12 | (12) |
| No | 5282 | (74) | 2236 | (71) | 5857 | (68) | 200 | (60) | 1636 | (72) | 1506 | (69) | 941 | (61) | 56 | (57) | |
| Unknown | 969 | (14) | 252 | (8) | 554 | (6) | 103 | (31) | 451 | (20) | 283 | (13) | 120 | (8) | 31 | (31) | |
| History of HIV infection | Yes | 109 | (2) | 184 | (6) | 905 | (11) | 13 | (4) | 22 | (1) | 45 | (2) | 127 | (8) | * | * |
| No | 5929 | (84) | 2685 | (85) | 7073 | (82) | 222 | (66) | 1785 | (78) | 1812 | (84) | 1291 | (83) | 65 | (66) | |
| Unknown | 1057 | (15) | 279 | (9) | 637 | (7) | 99 | (30) | 469 | (21) | 311 | (14) | 134 | (9) | * | * | |
Note. Hemophilia severity is defined based on the level of factor activity circulating in the blood. In this table, “mild” is defined as greater than 5% 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 baseline factor activity is missing or unknown. Males and females with factor VIII and IX deficiency are classified the same. Beginning with the September 2020 Report, data for persons with baseline factor activity >=40% are displayed separately from persons with greater than 5% and less than 40% activity.
† The HTC Population Profile contains 44 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.
* 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.