In 2 studies that have reported monogenic defects in children with Evans syndrome (autoimmune haemolytic anaemia and thrombocytopenia), more than 2/3rd patients were found to have pathogenic variants in various genes especially in the genes that also predispose to CVID such as LRBA and CTLA-4 (77, 78). Here, CTLA4 is linked to autoimmune hemolytic anemia.