Primary Immunodeficiencies Flashcards
name the 4 main components of the immune system and their immune mechanism
B cells and antibodies (humoral, specific immunity)
T cells (cellular, specific immunity)
Phagocytes (innate immunity)
complement system (innate immunity)
what are primary immunodeficiencies?
- Group of > 300 rare, chronic disorders in which part of the body’s immune system is missing or functions improperly
- Caused by single genetic defects
- May affect a single part of the immune system or more components of the immune system
main causes of primary immunodeficiencies in europe
predominately antibody disorders predominantely T cell disorders phagocyte disorders other well defined PIDs complement deficiencies auto-immune and immune dysregulation syndromes auto-inflammatory syndromes unclassified
what are secondary immunodeficiences
- Components of the immune system itself are all present and functional.
- Acquired diseases affecting the immune system and/or treatments negatively influencing the immune system.
- Caused by environmental/iatrogenic insults.
- Most well-known examples are HIV infection and patients treated for malignancies.
- Much more common than primary immunodeficiencies.
causes of secondary immunodeficiencies: environmental
malnutrition
trauma
burns
causes of secondary immunodeficiencies: disease
infection diabetes renal failure asplenia malignancies (leukaemia, lymphoma)
causes of secondary immunodeficiencies: iatrogenic
surgery
splenectomy
drugs (immunosuppressive, antirheumatic, antiepileptic)
3 categories of primary immunodeficiencies
antibodies deficiencies
cellular immunodeficiencies
innate immune disorders
primary immunodeficiencies: antibody deficiency
characterised by a deficiency of one or more (sub)classes of antibodies e.g. IgG, IgA, IgM, IgG2 due to defective B cells
or
absence of mature B cells
primary immunodeficiencies: cellular immunodeficinces
characterised by impaired T cell funciton or the absence of normal t cells
primary immunodeficiencies: innate immune disorders
defects in phagocyte function
complement deficiencies
absence or polymorphisms in pathogen recognition receptors
infections in primary immunodeficiencies: antibody deficiencies
recurrent bacterial infections of the upper and/or lower RT
s. pneumoniae, H. influenzae
infections in primary immunodeficiencies: cellular immunodeficiencies
unisal or opportunistic infections often combined with failure to thrive
pneumocystic jirovecii, CMV pneumonia
infections in primary immunodeficiencies: innate immune disorders
defects in phagocyte function: s. aureus (sepsis, skin lesions, abscesses internal organs) aspergillus infections (lung, bones, brain)
complement deficiencies
n. miningitidis
consequences of neutrophil defects
- Absence of neutrophils > congenital neutropenia
- Adhesion > leucocyte adhesion defect
- Recognition and Phagocytosis > deficiencies of PRR
- Intracellular Killing > chronic granulomatous disease