WASP expression correlates with clinical characteristics
. | WASP . | . | . | |
|---|---|---|---|---|
| Characteristics . | Positive . | Negative . | P . | |
| No. patients | 27 | 23 | — | |
| Total patient-years observed | 323 | 144 | — | |
| Clinical phenotype, n | ||||
| XLT phenotype (scores 1, 2, 5) | 21 | 2 | — | |
| WAS phenotype (scores 3, 4, 5) | 6 | 21 | — | |
| Score 5 | 7 | 8 | — | |
| XLT phenotype | 5 | 1 | — | |
| WAS phenotype | 2 | 7 | — | |
| 5A | 7 | 3 | — | |
| 5M | 0 | 3 | — | |
| 5A/M | 0 | 2 | — | |
| Age distribution, y | ||||
| Alive, n | 26 | 17 | — | |
| Mean, y | 12.1 | 6.1 | < .0001 | |
| Range, y | 1-61 | 1-15 | — | |
| Older than 15 y, n | 8 | 1 | — | |
| Dead, n | 1 | 6 | — | |
| Mean, y | 8 | 6.8 | — | |
| Range, y | 8 | 1-16 | — | |
| Older than 15 y, n | 0 | 1 | — | |
| Age on first visit, mo | ||||
| Mean ± SD (n) | 4.4 ± 5.1 (22) | 2.3 ± 3.2 (22) | .19* (—) | |
| Range | 0.1-18 | 0.1-11 | — | |
| Initial symptoms, n | ||||
| Thrombocytopenia-related | 25 | 20 | — | |
| No symptoms | 6 | 4 | — | |
| Petechiae | 16 | 3 | — | |
| Hematorrhea | 2 | 13 | — | |
| Intracranial hemorrhage | 1 | 0 | — | |
| Eczema | 2 | 3 | — | |
| Recurrent infection | 0 | 0 | — | |
. | WASP . | . | . | |
|---|---|---|---|---|
| Characteristics . | Positive . | Negative . | P . | |
| No. patients | 27 | 23 | — | |
| Total patient-years observed | 323 | 144 | — | |
| Clinical phenotype, n | ||||
| XLT phenotype (scores 1, 2, 5) | 21 | 2 | — | |
| WAS phenotype (scores 3, 4, 5) | 6 | 21 | — | |
| Score 5 | 7 | 8 | — | |
| XLT phenotype | 5 | 1 | — | |
| WAS phenotype | 2 | 7 | — | |
| 5A | 7 | 3 | — | |
| 5M | 0 | 3 | — | |
| 5A/M | 0 | 2 | — | |
| Age distribution, y | ||||
| Alive, n | 26 | 17 | — | |
| Mean, y | 12.1 | 6.1 | < .0001 | |
| Range, y | 1-61 | 1-15 | — | |
| Older than 15 y, n | 8 | 1 | — | |
| Dead, n | 1 | 6 | — | |
| Mean, y | 8 | 6.8 | — | |
| Range, y | 8 | 1-16 | — | |
| Older than 15 y, n | 0 | 1 | — | |
| Age on first visit, mo | ||||
| Mean ± SD (n) | 4.4 ± 5.1 (22) | 2.3 ± 3.2 (22) | .19* (—) | |
| Range | 0.1-18 | 0.1-11 | — | |
| Initial symptoms, n | ||||
| Thrombocytopenia-related | 25 | 20 | — | |
| No symptoms | 6 | 4 | — | |
| Petechiae | 16 | 3 | — | |
| Hematorrhea | 2 | 13 | — | |
| Intracranial hemorrhage | 1 | 0 | — | |
| Eczema | 2 | 3 | — | |
| Recurrent infection | 0 | 0 | — | |
WASP-positive numbers represent WASP detectable by Western blot. WASP-negative numbers represent WASP found absent or truncated by Western blot. — indicates not applicable.
Not significant.