Table 3.
Demographics of patients with confirmed autoimmune lymphoproliferative syndrome
| Patient Characteristics | All Patients with ALPS (n = 234) | Patients with ALPS without Lung Lesions (n = 216) | Patients with ALPS with Lung Lesions (n = 18) | P Value |
|---|---|---|---|---|
| Sex |
0.3069 | |||
| Male | 151 (64.5%) | 137 (63.4%) | 14 (77.8%) | |
| Female | 83 (35.5%) | 79 (36.6%) | 4 (22.2%) | |
| Median age at presentation, yr (range) | 14 (1–63) | 13 (1–63) | 14 (6–39) | |
| ALPS subclass |
0.0000 | |||
| ALPS-U | 45 (19.2%) | 31 (14.4%) | 14 (77.8%) | |
| ALPS-FAS | 163 (69.6%) | 160 (74.1%) | 3 (16.7%) | |
| ALPS-FASLG | 1 (0.4%) | 0 (0.0%) | 1 (5.6%) | |
| ALPS-sFAS | 19 (8.1%) | 19 (8.8%) | 0 (0.0%) | |
| ALPS-CASP10 | 6 (2.6%) | 6 (2.8%) | 0 (0.0%) | |
Definition of abbreviations: ALPS = autoimmune lymphoproliferative syndrome; ALPS-CASP10 = ALPS with mutations in caspase 10; ALPS-FAS = ALPS with homozygous or heterozygous mutations in FAS; ALPS-FASLG = ALPS with mutations in the FAS ligand; ALPS-sFAS = ALPS with somatic mutations in FAS; ALPS-U = autoimmune lymphoproliferative syndrome with undetermined genetic defect.
Data are presented as count (percent) or median (range).