Several ALPS-like patients with elevated DNT suffering from CTLA4 haploinsufficiency, LRBA, TPP2, ADA2 and IL2RB deficiencies, RALD syndrome or CARD11 GOF were treated and responded to this drug (31, 44, 55, 61, 71, 83, 96–98) (Table 1). Here, ADA2 is linked to autoimmune lymphoproliferative syndrome.