STEP1_Immunology

Question 1

What is graft v. host rejectio/n

Answer 1

Occurs when grafted T-cells proliferate and reject the host cells will foreign proteins (antigens).

Question 2

What type of rejection occurs immediately after transplant, via anti-donor antibodies?

Answer 2

Hyperacute rejection.

Question 3

What is an acute rejection?

Answer 3

Cell-mediated reaction via cytotoxic T-cells that react against foreign MHC.

~weeks post-transplant.

Question 4

What type of rejection occurs months to year post transplant via antibody-mediated vascular damage?

Answer 4

Chronic Rejection

Question 5

Bruton’s agammaglobulinemia and SCID are what type of immune disease?

Answer 5

B-cell def

Question 6

Thymic aplasia (DiGeorge) and Chronic Mucocutaneous Candidiasis are what type of immune disease?

Answer 6

T-cell Def

Question 7

SCID, Wiskott-Aldrich, and Ataxia-Telangiectasia are what type of immune disease?

Answer 7

B and T cell def.

Question 8

List the common phagocytic def. (6)

Answer 8

1. Chronic Granulomatous Disease
2. Chediak-Higashi
3. Job’s Syndrome
4. Leukocyte adhesion def.
5. Hyper-IgM syndrome
6. IL-12 Receptor def.

Question 9

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

Answer 9

Bruton’s agammaglobulinemia

Question 10

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

Answer 10

Selective Immunoglobulin Def

Question 11

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

Answer 11

Thymic Aplasia

Question 12

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)

Answer 12

SCID

Question 13

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

Answer 13

WISKOTT-­ALDRICH SYNDROME

Question 14

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)

Answer 14

CHRONIC GRANULOMATOUS DISEASE

Question 15

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

Answer 15

CHEDIAK­-HIGASHI DISEASE

Question 16

Name that disease:

T‐cells fail to produce γ‐interferon, thus PMN’s fail to respond, results in: ‐
Eczema, staph abscesses, and elevated IgE

Answer 16

JOB’S SYNDROME

Question 17

Name that disease:

There is a defect in the LFA‐1 adhesion proteins on the phagocytes, results in severe early life pyogenic infections.

Answer 17

LEUKOCYTE ADHESION DEFICIENCY SYNDROME

Question 18

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

Answer 18

HYPER­-IgM SYNDROME

Question 19

What is the Serum Ca+ levels of DiGeorge pt?

Answer 19

HypOcalcemia d/t to hypOparathyroisim

Question 20

Gonandal dysgenesis is a feature of what congenital syndrome?

Answer 20

Turners

Question 21

Decrease expression of CD40L == ??

Answer 21

Hyper IgM syndrome;

low levels of IgA, IgE, IgG

Question 22

What is the presentation of autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) syndrome?

Answer 22

hypoparathyroidism, adrenal insufficiency, chronic mucocutaneous candidiasis, and skin dystrophy

Question 23

autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) syndrome is d/t to what?

Answer 23

monogenic mutation of AIRE genes (autoimmune regulators); allows escape of self-reactice T-cells from thymus that expend into the periphery and induce autoimmunity.

Question 24

What does AIRE do?

Answer 24

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.

Question 25

What AI disease can follow after an infection (like with chlamydia) or diarrhea illness?

Answer 25

Reactive Arthritis

Question 26

omphalitis, delayed separation of the umbilical cord stump, is classic S.Sx for what?

Answer 26

leukocyte adhesion deficiency (LAD) type 1.

also need to have leukocytosis, but duh…

Question 27

Rotavirus vaxx is what type?

Answer 27

live-attenuated.

just like:
MMR,
Sabin polio
intranasal influenza
varicella
yellow-fever