TABLE 1.

An overview of the major clinical phenotypes associated with defects in each of the primary immune system components

Major clinical phenotypesa associated with defects in:
Adaptive immune system (combined PIDs)		Innate immune system
B cell defectsb	T cell defectsc	Phagocyte defectsb	Complement defectsd
Recurrent bacterial sinopulmonary infections	Failure to thrive (FTT)	Multiple or recurrent soft tissue abscesses, lymphadenitis	Angioedema of face, extremities, and/or GI tract
Sepsis (bacterial)	Opportunistic infections (e.g., PJP)	Soft tissue granulomas or infections with catalase-positive organisms or certain fungi (e.g., Aspergillus)	Pyogenic infections
Recurrent bacterial sinopulmonary infections or bacterial sepsis with encapsulated organisms	Fungal infections	Improper wound healing	Recurrent or systemic neisserial infections
Recurrent or chronic gastroenteritis (e.g., with enteroviruses or Giardia)	Recurrent, severe, or unusual viral infections	Gingivitis and periodontitis, chronic	Atypical hemolytic-uremic syndrome and/or thrombotic microangiopathy
Bronchiectasis with no clear cause	Graft-vs-host-type phenotype with elevated liver function tests, chronic GI manifestations (diarrhea)	Ulcerations of the mucosa
Enteroviral meningoencephalitis, usually chronic		Delayed separation of the umbilical cord

^aPIDs, primary immunodeficiencies: GI, gastrointestinal; PJP, Pneumocystis jirovecii pneumonia.

^bFound by flow cytometry and genetic testing.

^cFound by flow cytometry and nongenetic molecular tests to assess thymic function and T cell repertoire diversity and by genetic testing.

^dFound primarily by serological testing and genetic testing.