Typically, APLAID seems to be caused by the c.2120C>A (p.Ser707Tyr) variant of PLCG2 located within the cSH2 domain, whereas PLAID is caused by PLCG2 in-frame deletions located in the cSH2 domain [1]. Here, PLCG2 is linked to PLCG2-associated antibody deficiency and immune dysregulation.