Patient characteristics of entire cohort (including MSD transplant recipients)
| Characteristics | Total, N = 925 | NH White, N = 477 | Hispanic, N = 201 | Black, N = 96 | Asian/PI, N = 43 | Native American, N = 42 | Other/Unknown, N = 66 | P value |
|---|---|---|---|---|---|---|---|---|
| Male sex | 641 (69.3%) | 345 (72.3%) | 130 (64.7%) | 73 (76.0%) | 32 (74.4%) | 14 (33.3%) | 47 (71.2%) | <0.001 |
| Median age at diagnosis, days (min–max) | 118 (−17–4,918) | 136 (0–4,918) | 100 (0–4,636) | 137 (0–907) | 48 (0–3,084) | 71 (1.0–526) | 106.5 (−17–2,269) | 0.09 |
| Median age at treatment, days (min–max) | 167 (0–7,067) | 176 (7–5,137) | 146 (13–4,783) | 188 (12–1,309) | 124 (17–3,631) | 128 (0–634) | 153 (16–7,067) | 0.55 |
| Age at treatment | 0.42 | |||||||
| <3.5 mo | 303 (32.8%) | 149 (31.2%) | 64 (31.8%) | 30 (31.3%) | 17 (39.5%) | 19 (45.2%) | 24 (36.4%) | |
| >3.5 mo | 622 (67.2%) | 328 (68.8%) | 137 (68.2%) | 66 (68.8%) | 26 (60.5%) | 23 (54.8%) | 42 (63.6%) | |
| Decade of treatment | <0.001 | |||||||
| 1982–1990 | 115 (12.4%) | 70 (14.7%) | 11 (5.5%) | 14 (14.6%) | 3 (7.0%) | 6 (14.3%) | 11 (16.7%) | |
| 1990–2000 | 224 (24.2%) | 132 (27.7%) | 37 (18.4%) | 19 (19.8%) | 6 (14.0%) | 16 (38.1%) | 14 (21.2%) | |
| 2000–2010 | 296 (32.0%) | 142 (29.8%) | 82 (40.8%) | 32 (33.3%) | 14 (32.6%) | 10 (23.8%) | 16 (24.2%) | |
| 2010–2020 | 290 (31.4%) | 133 (27.9%) | 71 (35.3%) | 31 (32.3%) | 20 (46.5%) | 10 (23.8%) | 25 (37.9%) | |
| Trigger | 0.03 | |||||||
| Family history | 264 (28.6%) | 134 (28.1%) | 59 (29.5%) | 21 (21.9%) | 14 (32.6%) | 14 (33.3%) | 22 (33.3%) | |
| Clinical illness | 515 (55.7%) | 288 (60.4%) | 98 (49.0%) | 56 (58.3%) | 20 (46.5%) | 22 (52.4%) | 31 (47.0%) | |
| NBS | 145 (15.7%) | 55 (11.5%) | 43 (21.5%) | 19 (19.8%) | 9 (20.9%) | 6 (14.3%) | 13 (19.7%) | |
| Missing | 1 (0.1%) | 0 (0.0%) | 1 (0.5%) | 0 (0.0%) | 0 (0.0%) | 0 (0.0%) | 0 (0.0%) | |
| Baseline infection | <0.001 | |||||||
| Active infection | 336 (36.9%) | 160 (34.3%) | 78 (39.2%) | 37 (38.5%) | 17 (40.5%) | 20 (47.6%) | 24 (37.5%) | |
| No infection | 271 (29.8%) | 143 (30.6%) | 60 (30.2%) | 27 (28.1%) | 18 (42.9%) | 8 (19.0%) | 15 (23.4%) | |
| Resolved infection | 209 (23.0%) | 115 (24.6%) | 50 (25.1%) | 26 (27.1%) | 4 (9.5%) | 6 (14.3%) | 8 (12.5%) | |
| Unknown infection status | 94 (10.3%) | 49 (10.5%) | 11 (5.5%) | 6 (6.3%) | 3 (7.1%) | 8 (19.0%) | 17 (26.6%) | |
| Missing | 15 (1.6%) | 10 (2.1%) | 2 (1.0%) | 0 (0.0%) | 1 (2.3%) | 0 (0.0%) | 2 (3.0%) | |
| Maternal engraftment | 164 (26.5%) | 71 (21.0%) | 48 (35.0%) | 15 (23.4%) | 11 (44.0%) | 9 (40.9%) | 10 (30.3%) | 0.004 |
| Maternal GVHD | 49 (5.4%) | 19 (4.1%) | 18 (9.0%) | 3 (3.2%) | 2 (4.8%) | 5 (12.2%) | 2 (3.1%) | 0.03 |
| Autoimmune cytopenia | 72 (7.9%) | 33 (7.0%) | 17 (8.5%) | 8 (8.3%) | 5 (11.9%) | 3 (7.3%) | 6 (9.4%) | 0.20 |
| Genotype | <0.001 | |||||||
| ADA | 55 (5.9%) | 33 (6.9%) | 9 (4.5%) | 7 (7.3%) | 0 (0.0%) | 2 (4.8%) | 4 (6.1%) | |
| DCLRE1C | 39 (4.2%) | 11 (2.3%) | 5 (2.5%) | 0 (0.0%) | 3 (7.0%) | 16 (38%) | 4 (6.1%) | |
| IL2RG | 274 (30%) | 157 (33%) | 41 (20%) | 41 (43%) | 21 (49%) | 4 (9.5%) | 10 (15%) | |
| IL7R | 67 (7.2%) | 26 (5.5%) | 31 (15%) | 3 (3.1%) | 1 (2.3%) | 1 (2.4%) | 5 (7.6%) | |
| JAK3 | 40 (4.3%) | 19 (4.0%) | 10 (7.2%) | 8 (8.3%) | 2 (4.7%) | 0 (0.0%) | 1 (1.5%) | |
| RAG1 | 80 (8.6%) | 41 (8.6%) | 17 (8.5%) | 6 (6.3%) | 3 (7.0%) | 6 (14%) | 7 (11%) | |
| RAG2 | 35 (3.8%) | 10 (2.1%) | 16 (8.0%) | 0 (0.0%) | 2 (4.7%) | 0 (0.0%) | 7 (11%) | |
| Other/unknown | 335 (36%) | 180 (38%) | 72 (36%) | 31 (32%) | 11 (26%) | 13 (31%) | 28 (42%) | |
| Characteristics | Total, | NH White, | Hispanic, | Black, | Asian/PI, | Native American, | Other/Unknown, | P value |
|---|---|---|---|---|---|---|---|---|
| Male sex | 641 (69.3%) | 345 (72.3%) | 130 (64.7%) | 73 (76.0%) | 32 (74.4%) | 14 (33.3%) | 47 (71.2%) | |
| Median age at diagnosis, days (min–max) | 118 (−17–4,918) | 136 (0–4,918) | 100 (0–4,636) | 137 (0–907) | 48 (0–3,084) | 71 (1.0–526) | 106.5 (−17–2,269) | 0.09 |
| Median age at treatment, days (min–max) | 167 (0–7,067) | 176 (7–5,137) | 146 (13–4,783) | 188 (12–1,309) | 124 (17–3,631) | 128 (0–634) | 153 (16–7,067) | 0.55 |
| 0.42 | ||||||||
| <3.5 mo | 303 (32.8%) | 149 (31.2%) | 64 (31.8%) | 30 (31.3%) | 17 (39.5%) | 19 (45.2%) | 24 (36.4%) | |
| >3.5 mo | 622 (67.2%) | 328 (68.8%) | 137 (68.2%) | 66 (68.8%) | 26 (60.5%) | 23 (54.8%) | 42 (63.6%) | |
| 1982–1990 | 115 (12.4%) | 70 (14.7%) | 11 (5.5%) | 14 (14.6%) | 3 (7.0%) | 6 (14.3%) | 11 (16.7%) | |
| 1990–2000 | 224 (24.2%) | 132 (27.7%) | 37 (18.4%) | 19 (19.8%) | 6 (14.0%) | 16 (38.1%) | 14 (21.2%) | |
| 2000–2010 | 296 (32.0%) | 142 (29.8%) | 82 (40.8%) | 32 (33.3%) | 14 (32.6%) | 10 (23.8%) | 16 (24.2%) | |
| 2010–2020 | 290 (31.4%) | 133 (27.9%) | 71 (35.3%) | 31 (32.3%) | 20 (46.5%) | 10 (23.8%) | 25 (37.9%) | |
| Family history | 264 (28.6%) | 134 (28.1%) | 59 (29.5%) | 21 (21.9%) | 14 (32.6%) | 14 (33.3%) | 22 (33.3%) | |
| Clinical illness | 515 (55.7%) | 288 (60.4%) | 98 (49.0%) | 56 (58.3%) | 20 (46.5%) | 22 (52.4%) | 31 (47.0%) | |
| NBS | 145 (15.7%) | 55 (11.5%) | 43 (21.5%) | 19 (19.8%) | 9 (20.9%) | 6 (14.3%) | 13 (19.7%) | |
| Missing | 1 (0.1%) | 0 (0.0%) | 1 (0.5%) | 0 (0.0%) | 0 (0.0%) | 0 (0.0%) | 0 (0.0%) | |
| Active infection | 336 (36.9%) | 160 (34.3%) | 78 (39.2%) | 37 (38.5%) | 17 (40.5%) | 20 (47.6%) | 24 (37.5%) | |
| No infection | 271 (29.8%) | 143 (30.6%) | 60 (30.2%) | 27 (28.1%) | 18 (42.9%) | 8 (19.0%) | 15 (23.4%) | |
| Resolved infection | 209 (23.0%) | 115 (24.6%) | 50 (25.1%) | 26 (27.1%) | 4 (9.5%) | 6 (14.3%) | 8 (12.5%) | |
| Unknown infection status | 94 (10.3%) | 49 (10.5%) | 11 (5.5%) | 6 (6.3%) | 3 (7.1%) | 8 (19.0%) | 17 (26.6%) | |
| Missing | 15 (1.6%) | 10 (2.1%) | 2 (1.0%) | 0 (0.0%) | 1 (2.3%) | 0 (0.0%) | 2 (3.0%) | |
| 164 (26.5%) | 71 (21.0%) | 48 (35.0%) | 15 (23.4%) | 11 (44.0%) | 9 (40.9%) | 10 (30.3%) | ||
| 49 (5.4%) | 19 (4.1%) | 18 (9.0%) | 3 (3.2%) | 2 (4.8%) | 5 (12.2%) | 2 (3.1%) | ||
| 72 (7.9%) | 33 (7.0%) | 17 (8.5%) | 8 (8.3%) | 5 (11.9%) | 3 (7.3%) | 6 (9.4%) | 0.20 | |
| 55 (5.9%) | 33 (6.9%) | 9 (4.5%) | 7 (7.3%) | 0 (0.0%) | 2 (4.8%) | 4 (6.1%) | | |
| 39 (4.2%) | 11 (2.3%) | 5 (2.5%) | 0 (0.0%) | 3 (7.0%) | 16 (38%) | 4 (6.1%) | ||
| 274 (30%) | 157 (33%) | 41 (20%) | 41 (43%) | 21 (49%) | 4 (9.5%) | 10 (15%) | ||
| 67 (7.2%) | 26 (5.5%) | 31 (15%) | 3 (3.1%) | 1 (2.3%) | 1 (2.4%) | 5 (7.6%) | ||
| 40 (4.3%) | 19 (4.0%) | 10 (7.2%) | 8 (8.3%) | 2 (4.7%) | 0 (0.0%) | 1 (1.5%) | ||
| 80 (8.6%) | 41 (8.6%) | 17 (8.5%) | 6 (6.3%) | 3 (7.0%) | 6 (14%) | 7 (11%) | ||
| 35 (3.8%) | 10 (2.1%) | 16 (8.0%) | 0 (0.0%) | 2 (4.7%) | 0 (0.0%) | 7 (11%) | ||
| Other/unknown | 335 (36%) | 180 (38%) | 72 (36%) | 31 (32%) | 11 (26%) | 13 (31%) | 28 (42%) | |
GVHD, graft-versus-host disease. Bold values indicate P values that met the threshold for statistical significance.
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