B&L Unit 2

Question 1

Type 1 Response

Answer 1

IgE mediated. Cross linkage of adjacent IgE on mast cells that activates release granules and histamine in first phase. 
Late phase occure 5 to 10 hours later. 
Major treatment is avoidance.  Epipen with epinephrin can help. 
Ex. allergic reaction
Ex. serum sickness?
Ex. Asthma 
Ex. Eczema 
Ex. Hay Fever

Question 2

Type 2 Response

Answer 2

Specific antibody mediated reaction via complement mediated damage or stimulatory hypersensitivity. Immunofluorescence as linear.
Ex. Myasthenia gravis
Ex. Graves disease
Ex. Hashimoto Thyroiditis

Question 3

Type 3 Response

Answer 3

Immune complexes trapped in basement membrane of blood vessels. Immunofluorescence is lumpy bumpy. Causes lumpy bumpy glomerulus.
Ex. Lupus
Ex. Initial Hypersensitivity Pneumonitis (Famer’s Lung)
Ex. Arthus Reaction
Ex. IgA Nephropathy
Ex. one shot serum sickness
Ex. Rheumatoid Arthritis (IgM against IgG)

Question 4

Type 4 Response

Answer 4

T cell mediated. 
Ex. TB Test
Ex. Contact Dermatitis (poison ivy)
Ex. Multiple Sclerosis
Ex. first set graft rejection
Ex. second set graft rejection
Ex. Sjogren Syndrome

Question 5

DiGeorge

Answer 5

Parathyroid should be carefully evaluated. Thymus does not form correctly. PreT cells have nowhere to mature. Results in lack of mature T cells.

Question 6

Myasthemia Gravis

Answer 6

Type 2 autoimmunity.

Autoantibody to acetylcholine receptor

Question 7

Systemic Lupus Erythematosis

Answer 7

Type 2 autoantibody to dsDNA. Anti-nuclear antibody (ANA) will be present. Type 3 lumpy bumpy complexes present in glomerulus.

Question 8

Rheumatoid Arthritis

Answer 8

Pollution is an important risk factor- it increases citrullinated proteins in the lung.
Mostly type 4 T cell damage.
Autoantibody is present (RF) but is not only factor. This is also type 2.

Question 9

Celiac Disease

Answer 9

Type 4.
HLA DQ2 and DQ8. Body accidentally phagocytoses gluten with tissue transglutaminase 2 and presents it on MHC 2s. T cell mediated immunity to gliadin (wheat peptide).

Question 10

Graves Disease

Answer 10

Type 2 hypersensitivity immune reaction. IgG antibody binds and activates TSH receptor and activates signaling pathway leading to hyperthyroidism.

Question 11

Chronic Frustrated Immune Response

Answer 11

Body makes immune response to something that doesn’t go away. Skewed toward inflammatory response and away from Tregs.
Ex. IBD, Crohn disease, celiac disease, chronic Be disease, psoriasis, periodontal disease

Question 12

Acute Promyelotic Leukemia

Answer 12

Tx: all trans retinoic acid (ATRA)

Question 13

Goodpasture Disease

Answer 13

Autoantibodies to the lung and kidney basement membranes (type 4 collagen).
Type 2 autoimmunity.
HLA DR2

Question 14

Autoimmune Thrombocytic Purpura (ATP)

Answer 14

Antibodies destroy platelets.

Type 2 autoimmunity.

Question 15

Autoimmune Hemolytic Anemia

Answer 15

Autoantigen to RBC antigen.

Question 16

Hashimoto Thyroiditis

Answer 16

Type 2 & 4.
Body creates antibodies to thyroid antigens.
Causes hypothyroidism.
Includes T cell mediated destruction of thyroid.

Question 17

Aire

Answer 17

Thymic transcription factor that affects gene expression that helps regulate and destroy T cells that attack self. Defect in aire leads to T cell mediated autoimmunity.

Question 18

Dressler Syndrome

Answer 18

Post- MI autoimmune response.
Type 2.
Specific autoantibody against epitope in pericardial or myocardial antigens.

Question 19

Chronic Be Disease

Answer 19

Type 4. Chronic frustrated immune response.

Question 20

SCID

Answer 20

No B or T cells made.
X-linked: defect in gamma chain of IL2 receptor.
Autosomal recessive: adenosine deaminase (ADA) deficiency.

Question 21

Brutons

Answer 21

No B cell development.

X linked: bruton’s tyrosine kinase (BTK) is normally important in B cell signaling. Causes agammaglobulinemia.

Question 22

X linked Hyper IgM

Answer 22

Defective class switching due to CD40/CD40L leads to high IgM with low IgG and IgA

Question 23

Common Variable Immunodefiency

Answer 23

Unknown cause.

It is difficult for these pts to trigger antibody production by B cells.

Question 24

Rheumatic Heart Disease

Answer 24

Autoantibody to laminin on heart valves.

Question 25

Major Basic Protein

Answer 25

Secreted by eosinophils (attracted by EFC-A, mixture of leukotrienes and prostaglandins, is secreted from mast cells) as part of late reaction to parasites.

Question 26

Job Syndrome (hyperIgE syndrome)

Answer 26

Autosomal dominant.
Develop hyperreactivity to own cells.
Inability to make IFN-gamma effectively leading to poor Th1 responses and predominance of Th2.

Question 27

Selective IgA Deficiency

Answer 27

increased frequency and severity of allergies

Question 28

Ataxia Teangiectasia

Answer 28

autosomal recessive

Question 29

Wiskott-Aldrich Syndrome

Answer 29

X linked
platelet and B cell deficiency
eczema

Question 30

Ankylosing Spondylitis

Answer 30

HLA-B27

Question 31

Type 1 Diabetes

Answer 31

Antibodies made to islet associated antigens.

Considered to be Type 4, because T cell mediated damage causes pathology.

Question 32

Autoimmune Polyglandular Syndrome Type 1

Answer 32

Mutation on Aire causes T cells to be selected inappropriately, allowing T cells to survive that are antibodies to Th17 cell and IL17 signaling factors.

• prevents attraction of M1 macrophages
• oral candidiasis common
• Addison’s disease (adrenal insufficiency) common
• hypoparathyroidism common
• diabetes common

Question 33

Chronic Granulomatous Disease (CGD)

Answer 33

• primary immunodeficiency that affects neutrohphil function
• characterized by recurrent life threatening fungal infections
• neutrophilia- but neutrophils cannot produce ROS (no NADPH oxidase) to kill pathogens
• formation of granulomas occurs
• X linked 65% of cases
• tx: prophylactic antibiotics, stem cell transplant, gene therapy