Rare Factor Deficiencies
Table 4. HTC Population Profile Patient Characteristics, Rare Factor Deficiencies (excluding Factor VIII and Factor IX), Data Reported From 1/1/2012 Through 3/31/2018
| FI | FII | FV | Factors V & VIII, combined |
FVII | FX | FXI | FXIII | Alpha-2 Antiplasmin Deficiency |
PAI-1 Deficiency |
||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| # | (%) | # | (%) | # | (%) | # | (%) | # | (%) | # | (%) | # | (%) | # | (%) | # | (%) | # | (%) | ||
| # of patients | 223 | (100) | 68 | (100) | 425 | (100) | 21 | (100) | 1670 | (100) | 185 | (100) | 1069 | (100) | 194 | (100) | 6 | (100) | 276 | (100) | |
| Age (years) | <2 | 8 | (4) | * | * | 13 | (3) | * | * | 31 | (2) | * | * | 40 | (4) | 10 | (5) | * | * | 7 | (3) |
| 2-10 | 57 | (26) | 16 | (24) | 54 | (13) | * | * | 475 | (28) | 37 | (20) | 172 | (16) | 38 | (20) | * | * | 51 | (18) | |
| 11-19 | 64 | (29) | 29 | (43) | 131 | (31) | * | * | 671 | (40) | 61 | (33) | 303 | (28) | 34 | (18) | * | * | 118 | (43) | |
| 20-44 | 61 | (27) | 14 | (21) | 142 | (33) | 6 | (29) | 337 | (20) | 45 | (24) | 261 | (24) | 80 | (41) | * | * | 65 | (24) | |
| 45-64 | 24 | (11) | * | * | 63 | (15) | * | * | 103 | (6) | 28 | (15) | 152 | (14) | 25 | (13) | * | * | 22 | (8) | |
| 65+ | 9 | (4) | * | * | 22 | (5) | * | * | 53 | (3) | * | * | 141 | (13) | 7 | (4) | * | * | 13 | (5) | |
| Sex† | Male | 93 | (42) | 27 | (40) | 164 | (39) | 11 | (52) | 831 | (50) | 80 | (43) | 492 | (46) | 96 | (49) | * | * | 96 | (35) |
| Female | 130 | (58) | 41 | (60) | 261 | (61) | 10 | (48) | 839 | (50) | 105 | (57) | 577 | (54) | 98 | (51) | * | * | 180 | (65) | |
| Ethnicity | Hispanic, Latino/a, or Spanish origin | 23 | (10) | * | * | 62 | (15) | * | * | 384 | (23) | * | * | 140 | (13) | * | * | * | * | * | * |
| Not Hispanic, Latino/a, or Spanish origin | 193 | (87) | 49 | (72) | 354 | (83) | 20 | (95) | 1247 | (75) | 137 | (74) | 913 | (85) | 158 | (81) | * | * | 222 | (80) | |
| Unknown | 7 | (3) | * | * | 9 | (2) | * | * | 39 | (2) | * | * | 16 | (1) | * | * | * | * | * | * | |
| Race | American Indian/Alaska Native | * | * | * | * | * | * | * | * | * | * | * | * | * | * | * | * | * | * | * | * |
| Asian | 7 | (3) | * | * | * | * | * | * | 52 | (3) | * | * | 41 | (4) | 20 | (10) | * | * | * | * | |
| Black or African American | 10 | (4) | * | * | 17 | (4) | * | * | 305 | (18) | 24 | (13) | 47 | (4) | 23 | (12) | * | * | 26 | (9) | |
| Native Hawaiian or other Pacific Islander | * | * | * | * | * | * | * | * | * | * | * | * | * | * | * | * | * | * | * | * | |
| White | 184 | (83) | 59 | (87) | 374 | (88) | 16 | (76) | 1187 | (71) | 133 | (72) | 928 | (87) | 142 | (73) | * | * | 224 | (81) | |
| More than one of these | * | * | * | * | * | * | * | * | 28 | (2) | * | * | 6 | (1) | * | * | * | * | * | * | |
| Unknown | 16 | (7) | * | * | 22 | (5) | * | * | 85 | (5) | 14 | (8) | 42 | (4) | * | * | * | * | 13 | (5) | |
| Insurance Status | Insured | 215 | (96) | 65 | (96) | 413 | (97) | 18 | (86) | 1636 | (98) | 182 | (98) | 1046 | (98) | 188 | (97) | * | * | 257 | (93) |
| Uninsured | * | * | * | * | 6 | (1) | * | * | 20 | (1) | * | * | 10 | (1) | * | * | * | * | 12 | (4) | |
| Unknown | * | * | * | * | 6 | (1) | * | * | 14 | (1) | * | * | 13 | (1) | * | * | * | * | 7 | (3) | |
| History of HCV infection | Yes | 9 | (4) | 6 | (9) | 10 | (2) | * | * | 19 | (1) | 8 | (4) | 25 | (2) | 11 | (6) | * | * | * | * |
| No | 146 | (65) | 41 | (60) | 297 | (70) | 13 | (62) | 1182 | (71) | 135 | (73) | 787 | (74) | 160 | (82) | * | * | 149 | (54) | |
| Unknown | 68 | (30) | 21 | (31) | 118 | (28) | * | * | 469 | (28) | 42 | (23) | 257 | (24) | 23 | (12) | * | * | * | * | |
| History of HIV infection | Yes | * | * | * | * | * | * | * | * | * | * | * | * | 6 | (1) | * | * | * | * | * | * |
| No | 152 | (68) | 47 | (69) | 305 | (72) | 14 | (67) | 1197 | (72) | 144 | (78) | 803 | (75) | 171 | (88) | * | * | 148 | (54) | |
| Unknown | * | * | * | * | * | * | * | * | * | * | * | * | 260 | (24) | * | * | * | * | * | * | |
Note. This table excludes individuals with Factor VIII deficiency (hemophilia A) and Factor IX deficiency (hemophilia B). “Factors V & VIII, combined” refers to an autosomal condition, which occurs when there is a defect in a gene on one of the first 22 pairs of chromosomes called autosomes. Hemophilia A and B are X-linked conditions, which occur when there is a defect on the 23rd pair of chromosomes which determine one’s sex.
† The HTC Population Profile contains 33 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.