Clinical and laboratory features of 12 healthy relatives with Fas defects
| Family* . | Race . | Gender . | Age (y) . | LN size (1 to 4+)† . | Splenomegaly/ splenectomy . | α/β DNTCs (%) (no./μL3)‡ . | Serum IgG (mg/dL)2-153 . | Serum IL-10 (pg/ml) . | Autoantibodies . | Autoimmunity . |
|---|---|---|---|---|---|---|---|---|---|---|
| 1 | W | F | 48 | 1+ | N/N | 11 (0.8) | NT | 26 | NT | NDf |
| 1 | W | F | 28 | 1+ | N/N | 18 (0.7) | 782 | 6 | − | ND |
| 2 | W | M | 70 | 1+ | N/N | 7 (0.5) | 688 | 8 | − | ND |
| 2 | W | F | 41 | 1+ | N/N | 8 (0.6) | 1010 | 4 | + | ND |
| 4 | W | F | 34 | 1+ | N/N | 36 (1.2) | NT | 25 | NT | ND |
| 4 | W | F | 62 | 1+ | N/N | 7 (0.3) | 1300 | 5 | − | ND |
| 4 | W | F | 37 | 1+ | N/N | 6 (0.3) | 844 | 0 | + | ND |
| 5 | W | F | 37 | 1+ | N/N | 9 (0.7) | 909 | 6 | + | ND |
| 20 | W | M | 45 | 1+ | N/N | 16 (1.2) | 1380 | 22 | − | ND |
| 20 | W | F | 10 | 1+ | N/N | 32 (2.0) | 1350 | 35 | − | ND |
| 30 | W | F | 38 | 1+ | N/N | 27 (2.1) | 1400 | 11 | + | ND |
| 31 | W | M | 33 | 1+ | N/N | 22 (2.1) | 1450 | 23 | − | ND |
| Family* . | Race . | Gender . | Age (y) . | LN size (1 to 4+)† . | Splenomegaly/ splenectomy . | α/β DNTCs (%) (no./μL3)‡ . | Serum IgG (mg/dL)2-153 . | Serum IL-10 (pg/ml) . | Autoantibodies . | Autoimmunity . |
|---|---|---|---|---|---|---|---|---|---|---|
| 1 | W | F | 48 | 1+ | N/N | 11 (0.8) | NT | 26 | NT | NDf |
| 1 | W | F | 28 | 1+ | N/N | 18 (0.7) | 782 | 6 | − | ND |
| 2 | W | M | 70 | 1+ | N/N | 7 (0.5) | 688 | 8 | − | ND |
| 2 | W | F | 41 | 1+ | N/N | 8 (0.6) | 1010 | 4 | + | ND |
| 4 | W | F | 34 | 1+ | N/N | 36 (1.2) | NT | 25 | NT | ND |
| 4 | W | F | 62 | 1+ | N/N | 7 (0.3) | 1300 | 5 | − | ND |
| 4 | W | F | 37 | 1+ | N/N | 6 (0.3) | 844 | 0 | + | ND |
| 5 | W | F | 37 | 1+ | N/N | 9 (0.7) | 909 | 6 | + | ND |
| 20 | W | M | 45 | 1+ | N/N | 16 (1.2) | 1380 | 22 | − | ND |
| 20 | W | F | 10 | 1+ | N/N | 32 (2.0) | 1350 | 35 | − | ND |
| 30 | W | F | 38 | 1+ | N/N | 27 (2.1) | 1400 | 11 | + | ND |
| 31 | W | M | 33 | 1+ | N/N | 22 (2.1) | 1450 | 23 | − | ND |