Table 1.
Clinical and immunological differences according to type of cytopenia.
| ES n 22 (%) | MAC n 18 (%) | p | |
|---|---|---|---|
| Presence of genetic variant | 11/22 (50) | 7/18 (39) | 0.48 |
| Females | 12/22 (55) | 5/18 (27) | 0.08 |
| Familiar history of autoimmunity | 7/22 (32) | 2/18 (11) | 0.11 |
| Associated immunological features a | 10/22 (45) | 14/18 (78) | 0.03* |
| ALPS phenotype at onset b | 10/22 (45) | 7/18 (39) | 0.67 |
| ALPS-like/CVID phenotype at onset | 10/22 (45) | 11/18 (61) | 0.32 |
| Alive | 20/22 (91) | 18/18 (100) | 0.18 |
| DNT c > 1.5% | 15/22 (68) | 15/18 (83) | 0.27 |
| Need for second-line therapy | 12/22 (55) | 8/18 (44) | 0.52 |
| Sequential cytopenia | 12/22 (55) | 10/18 (56) | 0.94 |
a
Celiac disease, presence of autoantibodies including antinuclear, anti-neutrophils, anti-neutrophil cytoplasmic, anti-smooth muscle, anti-thyreoperoxydase, anti-thyroglobulin, lupus anticoagulant, anti-ADAMTS13, extractable nuclear antigen, cold agglutinins.
b
Presence of required and accessory diagnostic criteria for ALPS.
c
Double-negative T cells (CD3+ CD4- CD8- TCRαβ+).
*Statistically significant.