PMC4506145_01
Immunological characteristics of patients with hypomorphic RAG deficiency and impaired antibody production against bacterial polysaccharide antigens.. Patient A...circles; patient B...triangles 22 (A) Impaired antibody production against bacterial polysaccharide antigens in RAG-deficient patients. Box-plots represent pn23-antibodies in 41 healthy individuals (B) Numbers of CD4+CD45RA+ (naive CD4) and CD8+CD45RA+CD62L+ (naive CD8) T cells given as percentage of total CD8- or CD4-positive T cells. Box-plots depict 50 healthy individuals (C) Kinetic of the total count of CD4+ T cells and naive CD4+CD45RA+ T cell depicted as average of both patients (D) Representative example of the distribution of TCR Vbeta genes 16, 17, 18, 22 (E) Numbers of switched memory B cells given as percentage of total CD19 B cells. Box-plots depict 50 healthy individuals (F) Numbers of CD21low B cells given as percentage of total CD19 B cells. Box-plots depict 50 healthy individuals (median,+; box, IQR; whiskers, q5-q95).
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IgG, mg/dl [normal range]: 697 [790-1700]) IgG1-subclass deficiency (serum IgG1, mg/dl [normal range]: 303 [500-880], normal levels of IgA, IgM, IgG2-4 and low pneumococcal IgG-antibodies despite the fact that recurrent pneumococcal infections had led to very high IgM-antibody titers (Fig 1A). IgG-anti-polysaccharide antibody deficiency was diagnosed and the rapid reappearance of pulmonary infections required resumption of two-weekly IVIG substitution therapy.