Immune Deficiencies Flashcards
True or False: Deficits in innate immunity do not affect adaptive immunity.
False. The innate immune system is required to initiate the adaptive response
Recurrent infections, multiple sites over time, less virulent organisms, unusual severity and difficult to treat are all signs of _
An immune deficiency
Lower Respiratory tract, GU tract, Blood are all usually [sterile/non-sterile]
Sterile
Joint fluids, Pleura and peritoneral cavities are all usually [sterile/non-sterile]
Sterile
Skin and hair are usually colonized by _ (2)
Staphylococci
Streptococci
The oral cavity is usually colonized by _ (6)
Anerobes Micro-aerophilic streptococci Grp A strep Pneumococci H. influenza N. Meningiditis
The GI tract is usually colonized by _ (3)
Gram negative rods
Anaerobes
micro-aerophilic streptococci
The vaginal vestibule is usually colonized by _ (2)
Lactobacilli
Gram negative rods
What is primary immuno-deficiency?
An immunodeficiency state that is genetic / inherited
What is secondary immunodeficiency?
An immunodeficiency that is due to some other underlying process
An antibody disorder implicates what type of cells?
B cells / TH2 cells
A cell mediated immunity disorder implicates what type of cell?
T cells
A phagocytic deficiency can involve what 3 types of cells?
PMNs
Monocytes
Macrophages
What age range carries the greatest risk of bacterial meningitis? Why
0-2 yrs old
Immatire B2 marginal zone lymphocytes
An antibody deficit involving IgM will lead to infection by what type of bug? (2)
Gram negative rod
Nisseria meningiditis
An antibody deficit involving IgM will lead to infection in what anatomical location?
Blood
An antibody deficit involving IgG will lead to infection by what type of bug? (3)
Encapsulated bacteria
Enterovirus
Giardia
An antibody deficit involving IgG will lead to infection in what anatomical location? (3)
Sinopulmonary tree
Meninges
GI
An antibody deficit involving IgA will lead to infection by what type of bug? (2)
Local flora
Giardia
An antibody deficit involving IgA will lead to infection in what anatomical location? (2)
Sinopulmonary tree
GI
A complement deficiency will lead to infection by what type of bug? (2)
Nisseria (systemic disease)
Encapsulated bacteria
A complement deficiency will lead to infection in what locations? (2)
Blood
Meninges
A defect in cellular immunity will lead to infection by what class of bacteria? What are 4 examples?
Intracellular bacteria Salmonella Legionella Listeria Tb
A defect in cellular immunity will lead to infection by what type of organism, other than intracell. bacteria? One example of each?
Fungi - cryptococcus
DNA virus - Herpes, CMV
Protozoa - Pneumocystis carinii, giardia, toxoplasmosis
Parasites - Strongyloides
A defect in PMNs leads to infection by what types of bugs? (3)
Staph Aureus
Gram negative rods - Pseudomonas
Fungi - Aspergillus
A defect in the spleen will lead to infection by what type of bug?
Encapsulated bacteria (especially s. pneumonia)
A defect in the spleen will lead to infection in what area?
Blood
A defect in the liver will lead to infection by what type of bug?
Gram negative rods
A defect in the liver will lead to infection in what area?
Blood
The most effective means to get rid of encapsulated bacteria involves the combination of _ and _
Antibody and complement
For effective serum bactericidal killing as well as phagocytic killing, what component of the complement system is essential?
C3
When PMNs are relatively high above 500, what is the risk of infection to the skin, blood, lungs? Rank highest to low
Blood
Lungs
Skin
When PMNs are relatively low, below 500, what is the risk of infection to the skin, blood, lungs? Rank highest to low
Skin
Lungs
Blood
(Think what changes is now skin is not as protected)
A defect in complement is characterized by recurrent GI infections, true or false
False
The TH2 axis is important for _ while the TH1 axis is important for _
B cell antibody production
Intracellular pathogen killing by CD8
When CD4 counts are below 100, what is the time line within which infection will occur?
3 days
45 yo female, 2 mon. post renal transplant, high dose steroids, hasn’t been right 5-7 days, mild disorientation, mild fever, mild stiff neck.
What is the significance of the steroids?
Steroids will block the adaptive immunity, block T cell expansion
45 yo female, 2 mon. post renal transplant, high dose steroids, hasn’t been right 5-7 days, mild disorientation, mild fever, mild stiff neck.
What is the site of infection?
Given the confusion, stiff neck, CNS (meninges)
45 yo female, 2 mon. post renal transplant, high dose steroids, hasn’t been right 5-7 days, mild disorientation, mild fever, mild stiff neck.
Why is her neck only mildly stiff?
Because of the immunosuppresants depressing her natural response
45 yo female, 2 mon. post renal transplant, high dose steroids, hasn’t been right 5-7 days, mild disorientation, mild fever, mild stiff neck.
What were the 3 options presented for infecting bugs?
Crytococcus
Listeria
TB
Adult AIDS, DiGeorge’s syndrome, INF gamma receptor deficiency, IL12 receptor deficiency are all examples of _
Cell mediated immune defects
2 yo male, recurrent pneumonia since birth, staphylococcus cellulitis, FHx + for uncle death at 7 yo from recurrent pneumonia
What is the likely cause of the recurrent infection?
Defective innate or cell mediated immunity
2 yo male, recurrent pneumonia since birth, staphylococcus cellulitis, FHx + for uncle death at 7 yo from recurrent pneumonia
Considering that the site of infection are skin and lungs, with staph, what is the likely defect?
PMNs
Chemotherapy, chronic granulomatous disease, leuocytes adhesion deficiency, hyper IgE (Jobs) syndrome and myeloperoxidase deficiency are all examples of _
cases of PMN deficiency
26 yo with recurrent pneumonia, rales in right axilla. No organisms cultured, infection cleared by antibiotics. Previous history of pulmonary histoplasmosis, cleared with antifungal therapy.
Where is the pneumonia located? What is the name for this condition?
Right middle lobe
Right middle lobe syndrome
26 yo with recurrent pneumonia, rales in right axilla. No organisms cultured, infection cleared by antibiotics. Previous history of pulmonary histoplasmosis, cleared with antifungal therapy.
Is this a fungal or bacterial re-infection?
Bacterial
26 yo with recurrent pneumonia, rales in right axilla. No organisms cultured, infection cleared by antibiotics. Previous history of pulmonary histoplasmosis, cleared with antifungal therapy.
Why is the history of histoplasmosis important?
Tells us how her barrier function was originally impaired
In addition to right middle lobe syndrome, recurrent cellulitis, kidney stones and retained foreign bodies can cause infections due to _
Impaired barrier function
17 yo female, 12hr worsening headache, stiff neck, vomiting. Brother had meningococcal meningitis 4 months ago.
Where is the infection? What is likely deficient? Organism?
CNS
Complement defect - terminal component
Meningococcus
Pneumococci and meningococci are both examples of _
Encapsulated bacteria
A defect in the classical complement pathway predisposes to _
infection with encapsulated bacteria
A defect in the alternative complement pathway predisposes to _
Meningococcal disease
A defect in the terminal complement pathway predisposes to _
Recurrent meningococcal disease
Hereditary angioedema, hemolytic uremic syndrom, paroxsymal nocturnal hemoglobinuria are all examples of disease where _
The control components of the complement pathway are mutated / non-functional
33yo female, acute fever, diffuse rash and shock. Asplenic since age 10.
What is the site of Infection? What is the likely culprit?
Blood Encapsulated bacteria (pnemococcus)
One way to help protect asplenic individual from infection is by the use of _
Conjugate vaccines
The importance of the liver in the immune system is _
It is required to filter portal blood
14 mth old boy, failure to thrive. Normal weight gain for 8 months, poor growth after that. Several infections of otitis media, a case of pneumonia and secondary pneumococcal bacteremia. 6 months antibiotics
What is the relevance of the thriving during the 1st 6 months?
Mothers antibodies were present
14 mth old boy, failure to thrive. Normal weight gain for 8 months, poor growth after that. Several infections of otitis media, a case of pneumonia and secondary pneumococcal bacteremia. 6 months antibiotics
What is the defect here? Where is the infection?
Humoral (B cell) defect
Sinopulmonary, Blood
14 mth old boy, failure to thrive. Normal weight gain for 8 months, poor growth after that. Several infections of otitis media, a case of pneumonia and secondary pneumococcal bacteremia. 6 months antibiotics
What type of bacteria is responsible for the infection, encapsulated or no capsule?
Encapsulated.
Bruton’s X linked agammaglobulinemia, X-linked hyper IgM syndrome, common variable immunodeficiency and selective IgA deficiency are all examples of _
Antibody deficiencies
3 month old, recurrent infection, failure to thrive. Recurrent diarrhea, pneumonia, bacteremia, eczema. Undetectable tonsils or lymph nodes.
What is the site of infection here?
Multiple, seems like everywhere
3 month old, recurrent infection, failure to thrive. Recurrent diarrhea, pneumonia, bacteremia, eczema. Undetectable tonsils or lymph nodes.
What are the likely infection agents?
Bacteria (intracell and extracell)
Viruses
3 month old, recurrent infection, failure to thrive. Recurrent diarrhea, pneumonia, bacteremia, eczema. Undetectable tonsils or lymph nodes.
What does all this point to?
Combined immune defect
Childhood AIDS, X-linked SCID, Janus Kinase deficiency, Adenosine deaminase deficiency, Omenn’s syndrome, Artemis nuclease deficiency, MHC deficiency are all examples of _
Combined immunodeficiency syndromes
The two major components of the reticuloendothelial system are _ and _
Liver (Kuppfer cells)
Spleen (sinusoids lined with macrophages)
Facial rash, photosensitivity, palmar erythema, nailbed inflammation, telangiectasia, IgM deposition in dermal / epidermal junction are consistent with what autoimmune disease?
Lupus
IFN gamma is necessary for activation of CD8 T cells. What other cytokine deficiency can resemble IFN gamma deficiency?
IL12
