Flashcards in Immunology - Primary Immunodeficiencies Deck (20):
What is the major hallmark of immune deficiency?
Recurrent infections. Slide 4
What does SPUR stand for and what does it do?
Highlights the clinical features suggestive of immunodeficiency. Alisw 5
Which classification of immunodeficiencies are more common and why?
Secondary as they are often acquired instead of being born with it (primary). Slide 6
What are some conditions associated with secondary immune deficiency?
Cancer of the immune system
Malnutrition. Slide 7
What are some organisms that are clinical features of phagocyte deficiencies?
Mycobacteria: TB & atypical
Fungi - candida & aspergilllus. Slide 13
What can go wrong with the movement of phagocytes/precursors in the bone marrow or tissues?
Maybe not be able to produce neutrophils or maturation of them. Slide 19
What is Recticular Dysgenesis and Kostmann Syndrome?
RD = Failure to produce neutrophils
KS = severe congenital neutropaenia (failure of neutrophil maturation). Slide 19
How would Kostmann Syndrome be managed?
Supportive treatments of antibiotics + anti-fungals and NEED definitive treatment of stem cell transplantation or G-CSF. Slide 21
If there was something wrong with the endothelial adhesion markers at sites of infection?
Leukocyte Adhesion Deficiency
Activation markers expressed on the endothelial cells aren't recognised and neutrophils don't exit the bloodstream. Slide 24
What would be the signs of Leukocyte Adhesion Deficiency?
Recurrent bacterial/fungal infections, high neutrophil counts and at the deep tissues where the site of infection is, there would be no pus. Slide 24
If there was a fault in recognition of the organism what could happen?
Defects in opsonin receptors may cause defective phagocytosis.
Defect of complement/antibody production decreases efficiency of opsonisation.
The recognition is just not as efficient. Slide 30+31
What could happen if there was a fault in phagocytosis and killing of the organism?
Chronic Granulomatous Disease
Deficiency of the intracellular killing mechanism of phagocytes.
Cannot generate O2 free radicals to kill the pathogen. Slide 37
What is Chronic Granulomatous Disease?
Inability to clear organisms and there is excessive inflammation due to that.
Leads to granuloma formation. Slide 38
What are the features of Chronic Granulomatous Disease?
Recurrent deep bacterial infections e.g. Staph., Aspergillus, mycobacterium.
Reccurent FUNGAL infections, granuloma formation, failure to thrive and lymphadenopathy. Slide 39
How can you test for Chronic Granulomatous Disease?
Test patients neutrophils against E coli, add H2O2 sensitive dye and if H2O2 is produced by the neutrophils the dye changes colour. Slide 40
How do you manage Chronic Granulomatous Disease?
Supportive treatment of antibiotics and anti-fungals and the definitive treatment of a stem cell transplantation. Slide 41
What happens if there is a fault in the activation of other components in the immune system?
E.g. if there is a deficiency in IL-12 which stimulates the TH1 cells to produce IFNgamma then the macrophage won't phagocytose as well causing increased susceptibility to intracellular bacteria e.g. M. TB and salmonella. Slide 45
What are some of the tests to investigate phagocyte function?
Full blood count.
Presence of pus.
Chemotactic and phagocytosis assays.
NBT. Slide 46
What is aggressive management of infection in patients with phagocyte deficiencies?
Surgical draining of abscesses. Slide 48