ALPS classification and distribution of different categories of patients seen and evaluated at National Institutes of Health Clinical Center as part of our current cohort
| ALPS classification . | Chronic LPD/splenomegaly . | Elevated αβ DNTs . | Apoptosis defect . | % (no.) of ALPS cases (N = 257) . |
|---|---|---|---|---|
| ALPS-FAS (germline mutation) | + | + | + | 72 (185) |
| ALPS-sFAS (somatic mutation) | + | + | ± | 0.5 (14) |
| ALPS-FASLG | + | + | + | < 1 (2) |
| ALPS-CASP10 | + | + | + | < 1 (4) |
| ALPS-U | + | + | ± | 20 (52) |
| ALPS-related apoptosis disorders | ||||
| Caspase-8 deficiency state | + | ± | ± | 2 |
| RALD (somatic NRAS and KRAS mutations) | + | ± | ± | 6 |
| ALPS classification . | Chronic LPD/splenomegaly . | Elevated αβ DNTs . | Apoptosis defect . | % (no.) of ALPS cases (N = 257) . |
|---|---|---|---|---|
| ALPS-FAS (germline mutation) | + | + | + | 72 (185) |
| ALPS-sFAS (somatic mutation) | + | + | ± | 0.5 (14) |
| ALPS-FASLG | + | + | + | < 1 (2) |
| ALPS-CASP10 | + | + | + | < 1 (4) |
| ALPS-U | + | + | ± | 20 (52) |
| ALPS-related apoptosis disorders | ||||
| Caspase-8 deficiency state | + | ± | ± | 2 |
| RALD (somatic NRAS and KRAS mutations) | + | ± | ± | 6 |
RALD indicates RAS-associated autoimmune leukoproliferative disorder.