Plasma FAS-L and IL-10 concentrations and DNT cell percentage in untreated and treated ALPS patients. (A) The plasma FAS-L concentrations (ng/mL) are shown for 54 patients with ALPS associated with FAS mutation (3 homozygous [ALPS-0], 41 heterozygous [ALPS-Ia], and 10 somatic [ALPS-Im]), 61 of their healthy relatives with FAS mutations (MPRs, n = 24) or without FAS mutations (HRs, n = 37), 12 ALPS-like patients presenting clinical and laboratory criteria for ALPS but in whom no causative mutations have been identified and 27 unrelated, age-matched, healthy controls (HCs). △ denotes values of patients treated with immunosuppressive drugs (n = 26: 3 ALPS-0; 8 ALPS-Ia; 7 ALPS-Im; and 8 ALPS-like), ● denotes values of untreated patients. (B) The plasma FAS-L concentrations (ng/mL) are shown for 1 ALPS-Im and 7 ALPS-Ia patients before and during immunosuppressive (IS) therapy (methotrexate [n = 1], pyrimethamine plus sulfadoxine [n = 1], azathioprine [n = 1], 6-mercaptopurin [n = 2], or azathioprine plus 6-mercaptopurin [n = 3]). (C, D) The plasma IL-10 concentrations (pg/mL) are shown for the same samples as in panels A and B, respectively. (E , F) The DNT cells percentages are shown for the same samples as in panels A and B, respectively. The wide horizontal bars denote the mean values for each group. P values for intergroup differences in the Mann-Whitney test are shown above the data. Dashed lines delineate the normal values (DNT cells [N ≤ 2% of T cells corresponding to N ≤ 120/mm³ before 2 years, N ≤ 70/mm³ between 2 and 6 years, N ≤ 50/mm³ between 6 and 12 years, and N ≤ 40/mm³ after 18 years], plasma FAS-L [N ≤ 0.2 ng/mL], and plasma IL-10 [N ≤ 20 pg/mL]).