ICD-11 definition : This is a rare disease with the absence of immunoglobulin class switch recombination,
the lack of immunoglobulin somatic hypermutations, and lymph node hyperplasia caused
by the presence of giant germinal centers.;
ICD-11 synonym : HIGM2 - [hyper-IgM syndrome type 2]; Hyper-IgM syndrome due to AICDA deficiency; Hyper-IgM syndrome due to AID deficiency; hyper-IgM syndrome type 2;
This is a rare disease with the absence of immunoglobulin class switch recombination,
the lack of immunoglobulin somatic hypermutations, and lymph node hyperplasia caused
by the presence of giant germinal centers.