Serum Ig levels

DTH reactions to common antigens

Reduced Normal

Normal or reduced Reduced

Morphology of lymphoid tissues

Absent or reduced follicles and germinal centers (B cell zones)

Usually normal follicles, may be reduced parafollicular cortical regions (T cell zones)

DTH, delayed-type hypersensitivity.

• Patients with immunodeficiencies are also susceptible to certain types of cancer. Many of these cancers appear to be caused by oncogenic viruses, such as the Epstein-Barr virus. An increased incidence of cancer is most often seen in T cell immunodeficiencies because, as discussed in Chapter 17, T cells play an important role in surveillance against oncogenic viruses and the tumors they cause.

• Paradoxically, certain immunodeficiencies are associated with an increased incidence of autoimmunity. The mechanism underlying this association is not known.

• Immunodeficiency may result from defects in lymphocyte development or activation or from defects in the effector mechanisms of innate and adaptive immunity. Immunodeficiency diseases are clinically and pathologically heterogeneous, in part because different diseases involve different components of the immune system.

In this chapter, we first describe congenital immunodeficiencies, including defects in components of the innate immune system, and defects in the humoral and cell-mediated arms of the adaptive immune system. We conclude with a discussion of acquired immunodeficiencies, with an emphasis on AIDS.

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