!IP_PIDs_Curs.pptVIP

Non Brunton-Agammaglobulinemia (NBA) - accounts for ~10% of the patients with XLA-phonotype -mutations in the miu heavy chain gene, lambda5, Igalpha, BLNK = blocks in proB or preB cell transition (earlier than in Btk mutations). - involved genes are autosomal, so both boys and girls are affected CVID, Common Variable Immunodeficiencies / acquired hypogammaglobulinemia (~1:30 000) -heterogenous group of disease, general impairment in humoral immune responses - profound decrease in IgG, IgA, and ~50% also lack IgM. There are circulating, mature, B cells, but no memory B cells. - recurrent bac