X-linked Agammaglobulinemia (XLA), BrutonísAgammaglobulinemia

Labs: Intermittent neutropenia, immunoglobulins<5% of normal, no B/plasma cells on flow cytometry, low or absent antibodies to immunization antigens

Treatment: IVIG, Aggressive Abx for infxns

also see B-cell defects

CHLA board review 2005