STEP1_Immunology Flashcards
What is graft v. host rejectio/n
Occurs when grafted T-cells proliferate and reject the host cells will foreign proteins (antigens).
What type of rejection occurs immediately after transplant, via anti-donor antibodies?
Hyperacute rejection.
What is an acute rejection?
Cell-mediated reaction via cytotoxic T-cells that react against foreign MHC.
~weeks post-transplant.
What type of rejection occurs months to year post transplant via antibody-mediated vascular damage?
Chronic Rejection
Bruton’s agammaglobulinemia and SCID are what type of immune disease?
B-cell def
Thymic aplasia (DiGeorge) and Chronic Mucocutaneous Candidiasis are what type of immune disease?
T-cell Def
SCID, Wiskott-Aldrich, and Ataxia-Telangiectasia are what type of immune disease?
B and T cell def.
List the common phagocytic def. (6)
- Chronic Granulomatous Disease
- Chediak-Higashi
- Job’s Syndrome
- Leukocyte adhesion def.
- Hyper-IgM syndrome
- IL-12 Receptor def.
Name that disease:
An x‐linked recessive defect in tyrosine kinase gene. results in: ‐
All Ig classes are decreased
‐ ‐
Get recurrent bacterial infections (after 6 months of age)
Only in boys
Bruton’s agammaglobulinemia
Name that disease:
A deficiency of a specific Ig class resulting in defect I isotype switching, results in: ‐
‐ ‐
Sinus infection Lung infections
Most commonly the deficient Ig is IgA
Selective Immunoglobulin Def
Name that disease:
There is a failure of the development in the thymus and parathyroid’s, due to failure of development of the 3rd and 4th pharyngeal pouch, results in: ‐ ‐ ‐
Tetany
Viral and fungal infections (recurrent)
Heart defects
Thymic Aplasia
Name that disease:
This condition leads to a defect in differentiation of the early stem‐cells, results in: ‐ Recurrent infections (viral, bacterial, fungal, and protozoal)
SCID
Name that disease:
Is an x‐linked defect in the ability to mount an IgM response to the capsular polysaccharides of bacteria, results in: ‐
‐ ‐ ‐
Low IgM levels High IgA levels
Normal IgE levels Classic triad of symptoms: Infections, Eczema, and Thrombocytopenic Purpura
WISKOTT-ALDRICH SYNDROME
Name that disease:
A lack of NADPH leads to a defect in neutrophil phagocytosis, results in: ‐
Succeptibility to opportunitic bacterial infections ‐
Diagnosis based on negative nitroblue tetrazolium dye reduction test
(CLASSIC USMLE QUESTION)
CHRONIC GRANULOMATOUS DISEASE
Name that disease:
A defect in microtubular function and lysosomal emptying of the phagocytic cells, results in: ‐
Recurrent pyogenic infections due to staph and strep
CHEDIAK-HIGASHI DISEASE
Name that disease:
T‐cells fail to produce γ‐interferon, thus PMN’s fail to respond, results in: ‐
Eczema, staph abscesses, and elevated IgE
JOB’S SYNDROME
Name that disease:
There is a defect in the LFA‐1 adhesion proteins on the phagocytes, results in severe early life pyogenic infections.
LEUKOCYTE ADHESION DEFICIENCY SYNDROME
Name that disease:
There is a defect in the CD40 ligand on the CD4 Th cells, leading to severe pyogenic infections early in life, results in: ‐ ‐
High IgM levels
Extremely low levels of IgG, IgA, and IgE
HYPER-IgM SYNDROME
What is the Serum Ca+ levels of DiGeorge pt?
HypOcalcemia d/t to hypOparathyroisim
Gonandal dysgenesis is a feature of what congenital syndrome?
Turners
Decrease expression of CD40L == ??
Hyper IgM syndrome;
low levels of IgA, IgE, IgG
What is the presentation of autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) syndrome?
hypoparathyroidism, adrenal insufficiency, chronic mucocutaneous candidiasis, and skin dystrophy
autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) syndrome is d/t to what?
monogenic mutation of AIRE genes (autoimmune regulators); allows escape of self-reactice T-cells from thymus that expend into the periphery and induce autoimmunity.
What does AIRE do?
A specialized population of cells in the thymus called the medullary epithelial cells (MECs), express and present a vast array of autoantigens to maturing T cells. T cells that bind with high affinity to these autoantigens are deleted. The AIRE gene product is a nuclear transcription factor that regulates the expression of autoantigens by MECs in the thymus. Mutations in AIRE allow the escape of self-reactive T cells from the thymus that expand in the periphery and induce autoimmunity.
What AI disease can follow after an infection (like with chlamydia) or diarrhea illness?
Reactive Arthritis
omphalitis, delayed separation of the umbilical cord stump, is classic S.Sx for what?
leukocyte adhesion deficiency (LAD) type 1.
also need to have leukocytosis, but duh…
Rotavirus vaxx is what type?
live-attenuated.
just like: MMR, Sabin polio intranasal influenza varicella yellow-fever
