Revised classification of ALPS
| Previous nomenclature . | Revised nomenclature . | Gene . | Definition . |
|---|---|---|---|
| ALPS type 0 | ALPS-FAS | FAS | Patients fulfill ALPS diagnostic criteria and have germline homozygous mutations in FAS. |
| ALPS type Ia | ALPS-FAS | FAS | Patients fulfill ALPS diagnostic criteria and have germline heterozygous mutations in FAS. |
| ALPS type Im | ALPS-sFAS | FAS | Patients fulfill ALPS diagnostic criteria and have somatic mutations in FAS. |
| ALPS type Ib | ALPS-FASLG | FASLG | Patients fulfill ALPS diagnostic criteria and have germline mutations in FAS ligand. |
| ALPS type IIa | ALPS-CASP10 | CASP10 | Patients fulfill ALPS diagnostic criteria and have germline mutations in caspase 10. |
| ALPS type III | ALPS-U | Unknown | Patients meet ALPS diagnostic criteria; however, genetic defect is undetermined (no FAS, FASL, or CASP10 defect). |
| Previous nomenclature . | Revised nomenclature . | Gene . | Definition . |
|---|---|---|---|
| ALPS type 0 | ALPS-FAS | FAS | Patients fulfill ALPS diagnostic criteria and have germline homozygous mutations in FAS. |
| ALPS type Ia | ALPS-FAS | FAS | Patients fulfill ALPS diagnostic criteria and have germline heterozygous mutations in FAS. |
| ALPS type Im | ALPS-sFAS | FAS | Patients fulfill ALPS diagnostic criteria and have somatic mutations in FAS. |
| ALPS type Ib | ALPS-FASLG | FASLG | Patients fulfill ALPS diagnostic criteria and have germline mutations in FAS ligand. |
| ALPS type IIa | ALPS-CASP10 | CASP10 | Patients fulfill ALPS diagnostic criteria and have germline mutations in caspase 10. |
| ALPS type III | ALPS-U | Unknown | Patients meet ALPS diagnostic criteria; however, genetic defect is undetermined (no FAS, FASL, or CASP10 defect). |