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Definition

A form of agammaglobulinemia, a primary immunodeficiency characterized by profoundly low or absent serum antibodies and low or absent circulating B-cells due to an early block of B-cell development. Affected individuals develop severe infections in the first years of life.

Acronym

AGM8

Synonyms

Agammaglobulinemia, autosomal dominant, due to TCF3 defect