Demographic characteristics and distribution of ALPS features
| Group . | No. . | Age at study . | Gender . | Race . | Number of ALPS features* . | |||||
|---|---|---|---|---|---|---|---|---|---|---|
| 0 . | 1 . | 2 . | 3 . | 4 . | 5 . | |||||
| Proband Ia | 31 | 9/8† (1-20) | 20m/11f (1.8:1) | 27W/3B/1Bi | 0 | 0 | 0 | 2 | 12 | 17 |
| M+/A+ | 28 | 28/28 (9-62) | 14m/14f (1:1) | 26W/2B | 0 | 0 | 0 | 9 | 9 | 10 |
| M+/A− | 42 | 41/42 (1-86) | 18m/24f (0.8:1) | 41W/1Bi | 17 | 20 | 4 | 1 | 0 | 0 |
| M−/A− | 65 | 35/33 (4-69) | 33m/32f (1:1) | 59W/6B | 50 | 13 | 2 | 0 | 0 | 0 |
| Proband II/III | 11 | 10/10 (1-20) | 7m/4f (1.8:1) | 9W/2B | 0 | 0 | 0 | 1 | 3 | 7 |
| Group . | No. . | Age at study . | Gender . | Race . | Number of ALPS features* . | |||||
|---|---|---|---|---|---|---|---|---|---|---|
| 0 . | 1 . | 2 . | 3 . | 4 . | 5 . | |||||
| Proband Ia | 31 | 9/8† (1-20) | 20m/11f (1.8:1) | 27W/3B/1Bi | 0 | 0 | 0 | 2 | 12 | 17 |
| M+/A+ | 28 | 28/28 (9-62) | 14m/14f (1:1) | 26W/2B | 0 | 0 | 0 | 9 | 9 | 10 |
| M+/A− | 42 | 41/42 (1-86) | 18m/24f (0.8:1) | 41W/1Bi | 17 | 20 | 4 | 1 | 0 | 0 |
| M−/A− | 65 | 35/33 (4-69) | 33m/32f (1:1) | 59W/6B | 50 | 13 | 2 | 0 | 0 | 0 |
| Proband II/III | 11 | 10/10 (1-20) | 7m/4f (1.8:1) | 9W/2B | 0 | 0 | 0 | 1 | 3 | 7 |
W indicates white; B, black; Bi, biracial; m, male; f, female.
Features in addition to impaired lymphocyte apoptosis seen in all M+ subjects: lymphadenopathy, splenomegaly, ≥ 1% α/β+-DNT cells, autoantibodies, autoimmune disease (only features documented in medical records were considered).
Mean/median age in years; (range).