10. Immunocompromised Host Flashcards
What is primary immunodeficiency (PID)?
Congenital immunodeficiency due to an intrinsic gene defect.
Could be missing protein missing cell or non-functional components.
What is secondary immunodeficiency?
Acquired immunodeficiency due to an underlying disease or treatment.
Could be due to decreased production or increased loss of immune components.
What acronym is used to identify when to suspect an immunodeficiency?
"SPUR" Severe Persistant Unusual - site or microorganism Recurrent
How many warning sites must be demonstrated to be diagnosed with PID?
2
What are some common causes of PID?
- Antibody deficiency
- Common variable immunodeficiencies (CVID)
- Combined T and T cell
- Phagocytic defects
Which PID is caused by phagocytic defects?
Chronic granulomatous disease - defective NADH oxidase
What information can be useful when trying to diagnose a PID?
Age of onset
Type of microbes and sites of infection
Presentation <6 months is likely to be defect in what?
T cell or phagocytic defect
Presentation 6 months- 5 years is likely to be a defect in what?
B cell/Antibody or phagocyte
What do antibody defects usually only present after around 6 months?
Maternal IgG antibodies are in the circulation for the first 6 months
Presentation >5 years is likely to be a defect in what?
B cell/antibody
Complement
or Secondary immune deficiency
What type of infections are characteristic of complement deficiency?
Meningitis, sepsis, arthritis (C5-C9) Pyogenic infections (C3)
What type of infections are characteristic of phagocyte defects?
Skin/mucous infections
Deep seated infections
Invasive fungal infections
Which fungal infection is associated with phagocyte defects?
Aspergillosis
What inheritance pattern does chronic granulomatous disease show?
Autosomal recessive
What infections are associated with antibody deficiency?
Sinorespiratory infections Arthopathies GI infections Malignancies Autoimmunity
Which type of PID is giardia lambia associated with?
Antibody
What X-linked disease causes defects in B cell development?
Bruton’s disease
What are the signs of T cell defects?
Failure to thrive
Deep skin and tissue accesses
Opportunistic infections
Death if not treated
What other immune cell will be affected by T cell defects?
B cells - T cells provide cytokine signals that stimulate isotype switching to IgG, so IgG levels will decrease.
Are viral and fungal infections associated with T or B cell deficiency?
T cell