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 3/31/2021

HTC Population Profile Patient Characteristics, Rare Factor Deficiencies (excluding Factor VIII and Factor IX)
FI FII FV Factors
V & VIII,
combined
FVII FX FXI FXIII Alpha-2
Antiplasmin
Deficiency
PAI-1
Deficiency
# (%) # (%) # (%) # (%) # (%) # (%) # (%) # (%) # (%) # (%)
# of patients 368 (100) 114 (100) 539 (100) 22 (100) 2515 (100) 270 (100) 1520 (100) 246 (100) 13 (100) 337 (100)
Age (years) <2 28 (8) * * 16 (3) * * 52 (2) * * 63 (4) 14 (6) * * 10 (3)
2-10 96 (26) 21 (18) 70 (13) * * 638 (25) 59 (22) 214 (14) 45 (18) * * 49 (15)
11-19 99 (27) 44 (39) 186 (35) 6 (27) 1050 (42) 79 (29) 404 (27) 45 (18) * * 132 (39)
20-44 87 (24) 29 (25) 168 (31) 7 (32) 540 (21) 70 (26) 431 (28) 102 (41) * * 94 (28)
45-64 38 (10) 12 (11) 73 (14) * * 146 (6) 43 (16) 217 (14) 25 (10) * * 31 (9)
65+ 20 (5) * * 26 (5) * * 89 (4) * * 191 (13) 15 (6) * * 21 (6)
Sex Male 164 (45) 43 (38) 206 (38) 11 (50) 1234 (49) 112 (41) 685 (45) 120 (49) * * 105 (31)
Female 204 (55) 71 (62) 333 (62) 11 (50) 1281 (51) 158 (59) 835 (55) 126 (51) * * 232 (69)
Ethnicity Hispanic, Latino/a, or Spanish origin 41 (11) * * 83 (15) * * 554 (22) 61 (23) 187 (12) * * * * 63 (19)
Not Hispanic, Latino/a, or Spanish origin 310 (84) 76 (67) 438 (81) 22 (100) 1845 (73) 196 (73) 1284 (84) 192 (78) * * 268 (80)
Unknown 17 (5) * * 18 (3) * * 116 (5) 13 (5) 49 (3) * * * * 6 (2)
Race American Indian/Alaska Native * * * * * * * * * * * * * * * * * * * *
Asian 15 (4) * * 14 (3) * * 74 (3) 20 (7) 54 (4) 24 (10) * * * *
Black or African American 18 (5) * * 20 (4) * * 454 (18) 29 (11) 68 (4) 24 (10) * * 35 (10)
Native Hawaiian or other Pacific Islander * * * * * * * * * * * * * * * * * * * *
White 293 (80) 94 (82) 460 (85) 15 (68) 1704 (68) 193 (71) 1293 (85) 177 (72) * * 273 (81)
More than one of these * * * * * * * * 44 (2) * * 10 (1) * * * * 6 (2)
Unknown 35 (10) 10 (9) 36 (7) * * 221 (9) * * 89 (6) * * * * 16 (5)
Insurance Status Insured 356 (97) 107 (94) 527 (98) 20 (91) 2453 (98) 267 (99) 1489 (98) 238 (97) * * 319 (95)
Uninsured * * * * * * * * 32 (1) * * 13 (1) * * * * * *
Unknown * * * * * * * * 30 (1) * * 18 (1) * * * * * *
History of HCV infection Yes 11 (3) 7 (6) 11 (2) * * 25 (1) 9 (3) 32 (2) 10 (4) * * * *
No 247 (67) 63 (55) 385 (71) 15 (68) 1872 (74) 200 (74) 1126 (74) 199 (81) * * 185 (55)
Unknown 110 (30) 44 (39) 143 (27) * * 618 (25) 61 (23) 362 (24) 37 (15) * * * *
History of HIV infection Yes * * * * * * * * * * * * 9 (1) * * * * * *
No 257 (70) 69 (61) 391 (73) 15 (68) 1897 (75) 210 (78) 1153 (76) 211 (86) * * 185 (55)
Unknown * * * * * * * * * * * * 358 (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 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.