Primary immunodeficiency states

Primary immunodeficiency states are heterogenous group of disorders that occur when there is an impairment of humoral or cell-mediated immunity in the absence of any recognized precipitating cause such as drug therapy or infective agent such as human immunodeficiency virus (HIV).

Epidemiology

The majority are diagnosed in children under the age of one, although milder forms may not be recognized until adulthood. 

Classification

They are broadly classified as disorders of adaptive immunity (i.e., T-cell, B-cell or combined immunodeficiencies) or of innate immunity (e.g., phagocyte and complement disorders. Over 260 conditions may exist 3.

Only some of the more recognized ones are listed below. 

T-cell (cellular) immunodeficiency
B-cell (antibody-mediated) immunodeficiency
CID (combined)
Disorders of innate immunity
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Article information

rID: 59495
System: Haematology
Section: Gamuts
Synonyms or Alternate Spellings:
  • Primary immunodeficiency state
  • Primary immunodeficiency disorder
  • Primary immunodeficiency disorders

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