Adenoid Hyperplasia in A Patient With A Rare Type of Hyper Immunoglobulin M Syndrome Due To Cd40 Deficiency

Cd40 deficiency yield to an autosomal recessive subtype of hyper-immunoglobulin m syndrome (hgim type 3), presenting with an almost identical clinical picture to x-linked cd40l deficiency (higm type 1) with profound t-cell dysfunction yielding to opportunistic infections as well as neutropenia, autoimmunity, and malignancy. we presented a girl with recurrent upper respiratory tract infections and lymphoid hyperplasia which was diagnosed with type 3 hyper igm syndrome due to cd40 gene mutation. otitis media with opportunistic germs and no evidence for an x-linked pattern of inheritance were diagnostic keys to type 3 hyper igm syndrome in our patient.