tokens: Among 246 patients with predominantly paediatric - onset PIDs from 2003 - 2015 , 21 [ Btk ( six ) , IL2RG ( four ) , WASP , CD40L , gp91 ( three each ) , gp47 , RAG2 ( one each ) ] and five [ CVID ( four ) , SCID ( one ) ] without identified mutations had SD before prophylactic treatment .