How Common Variable Immune Deficiency has Changed Over Six Decades
Cunningham-Rundles C., Chapel H.
© 2014 Elsevier Inc. All rights reserved. Starting about 60 years ago, a number of reports appeared that outlined the severe clinical course of a few adult subjects with profound hypogammaglobulinemia. Puzzled by the lack of family history and adult onset of symptoms in most, the name "acquired" hypogammaglobulinemia was given, but later altered to the current name common variable immune deficiency (CVID). Pathology reports remarked on the loss of lymph node architecture and paucity of plasma cells in these subjects. While characterized by reduced serum IgG and IgA and often IgM, and thus classified among the B-cell defects, numbers of cellular defects have been recognized over time. In the past, severe respiratory tract infections commonly led to a shortened life span but the widespread availability of immune globulin concentrates for the last 25 years has dramatically lowered the morbidity and mortality noted in earlier reports.