acronym for Autosomic Recessive AGammaglobulinemia

(agammglobulinemia unlinked to gender)

Extremely rare: it accounts for 15 % of the congenital agammaglobulinemias. Autosomal recessive transmission. There are 8 types the description of which  is often limited to a family or a few individuals.

• AGM1: mutation of the IGHM gene (14q32.33) [MIM 601 495]
• AGM2: mutation of the IGLL1 gene (22q11.23) [MIM 613 500]
• AGM3: mutation in the CD79A gene (13q13.2) [MIM 613 501]
• AGM4: mutation of the BLNK gene (10q24.1) [MIM 613 502]
• AGM5: mutation of the LRRC8 gene (9q24.11) [MIM 613 506]
• AGM6: mutation of the CD79B gene (17q23.3) [MIM 612 692]
• AGM7: mutation of the PIK31 gene (5q13.1) [MIM 615 214]
• AGM8: mutation of the TCF3 gene (19p13.3) [MIM 616 941]

Clinical presentation similar to Bruton's disease: first signs in very young children, in the first year of life: repeated infections, especially respiratory ones and more rarely, digestive and cutaneous ones, especially of bacterial origin.

Treatment: regular administration of IV immunoglobulins.

Anesthetic implications: 

strict aseptic rules, difficult peripheral venous access

References : 

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Updated: December 2021