Among inhibitory receptors, the frequency of total NK cells expressing LAG-3 was increased in STS patients (2%, IQR: 0.4–3, N = 7) compared with CTRL (0.07%, IQR: 0.03–0.15, N = 11, p = 0.01), possibly reflecting a state of functional exhaustion. Here, LAG3 is linked to telomere syndrome.