Table 5.
Clinical and immunological differences according to variants.
| Tot (%) | Patients with variants (18/40, 45%) | Patients without variants (22/40, 55%) | p | |
|---|---|---|---|---|
| Females | 17/40 (43) | 10/18 (56%) | 7/22 (32%) | 0.13 |
| Familiar history of autoimmunity | 9/40 (23) | 5/18 (28%) | 4/22 (18%) | 0.47 |
| Associated immunological features a | 24/40 (60) | 9/18 (50%) | 15/22 (68%) | 0.22 |
| ALPS phenotype at onset b | 17/40 (43) | 10/18(56%) | 7/22 (32%) | 0.13 |
| DNT c >1.5% | 30/40 (75) | 15/18 (83%) | 15/22 (68%) | 0.27 |
| Signs of lymphoproliferation d | 27/40 (68) | 13/18 (72%) | 14/22 (64%) | 0.56 |
| Need for second-line therapy | 28/40 (70) | 14/18 (78%) | 14/22 (64%) | 0.33 |
| Sequential cytopenia | 22/40 (55) | 10/18 (56%) | 12/22 (55%) | 0.94 |
a
Celiac disease, presence of autoantibodies including antinuclear, anti-neutrophils, anti-neutrophil cytoplasmic, anti-smooth muscle, anti-thyroperoxydase, anti-thyeroglobulin, 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+).
d
Chronic (> 6 months), non-malignant, non-infectious lymphadenopathy or splenomegaly or both.