In the present cohort, a patient was identified with a novel heterozygous nonsense variant in NF-κB2 (located in the ankyrin repeat–containing domain) and presented with ITP at age 3, which subsequently developed into Evans' syndrome nearly 6 months later, with numerous positive indicators of autoimmunity such as antinuclear. Here, NFKB2 is linked to autoimmune thrombocytopenic purpura.