Overview

Patients with immunodeficiency typically experience stereotypic patterns of recurrent infection, which provide clues regarding which portion of the immune system is affected. Primary immunodeficiency occasionally presents for the first time in adulthood. Affected patients may also have autoimmune disease and suffer higher rates of malignancies, as these disorders are also manifestations of abnormal immune function. A history of one or more of these types of disorders in a patient with recurrent infections should raise suspicion of an underlying immunodeficiency. 

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Defects in immunoglobulins and/or complement proteins

Recurrent sinopulmonary infections, chronic gastrointestinal infections, bacteremia, and/or meningitis are associated with defects in immunoglobulins and/or complement proteins.

 

Granulocyte (neutrophil) defects 

Recurrent invasive skin and soft tissue infections, especially focal abscesses requiring incision and drainage, are associated with granulocyte (neutrophil) defects. Characteristic organisms include catalase-positive organisms, such as S. aureus, gram-negative bacilli, Aspergillus, and Nocardia. 

 

Defects in cell-mediated immunity 

Progressive infections with ordinarily "benign" viruses, opportunistic intracellular pathogens, or fungi suggest defective cell-mediated immunity, particularly defects of T cells. Typical micro-organisms include cytomegalovirus, Epstein-Barr virus or other herpes viruses, mycobacteria, and fungi (Candida, Cryptococcus, and Pneumocystis). Natural killer (NK) cell defects also present with severe and fulminant herpes virus infections, although these conditions are rare.