Diagnostic criteria for ALPS as defined in 1999
| Required criteria |
| Chronic nonmalignant lymphadenopathy and/or splenomegaly |
| Increased peripheral CD3+TCRαβ+CD4−CD8− (DNT) cells |
| Lymphocyte apoptosis defect |
| Supporting criteria |
| Family history of ALPS |
| Characteristic histopathology |
| Autoimmune manifestations |
| Required criteria |
| Chronic nonmalignant lymphadenopathy and/or splenomegaly |
| Increased peripheral CD3+TCRαβ+CD4−CD8− (DNT) cells |
| Lymphocyte apoptosis defect |
| Supporting criteria |
| Family history of ALPS |
| Characteristic histopathology |
| Autoimmune manifestations |