Common variable immunodeficiency
  • Heterogenous group of disorders characterized by hypogammaglobulinemia (usually all immunoglobins but sometimes just IgG)
  • Term refers to disorders for which no primary defect or secondary cause can be identified
  • Three types:
    • Predominant intrinsic B-cell defect
    • Predominant T-cell disorder with T-helper cell defect or T-supressor cell surplus
    • Autoantibodies to T or B cells
  • Hyperplastic B-cell areas
  • Patients suffer recurrent bacterial infections and parasitic infections (Giardia common)
  • High frequency of autoimmune disorders
  • Sometimes progressing to lymphoid malignancy
  • spruelike illness and noncaseating granulomas in liver
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