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Flashcards in B&L Unit 2 Deck (33)
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1
Q

Type 1 Response

A
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
2
Q

Type 2 Response

A

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

3
Q

Type 3 Response

A

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)

4
Q

Type 4 Response

A
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
5
Q

DiGeorge

A

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

6
Q

Myasthemia Gravis

A

Type 2 autoimmunity.

Autoantibody to acetylcholine receptor

7
Q

Systemic Lupus Erythematosis

A

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

8
Q

Rheumatoid Arthritis

A

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.

9
Q

Celiac Disease

A

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).

10
Q

Graves Disease

A

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

11
Q

Chronic Frustrated Immune Response

A

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

12
Q

Acute Promyelotic Leukemia

A

Tx: all trans retinoic acid (ATRA)

13
Q

Goodpasture Disease

A

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

14
Q

Autoimmune Thrombocytic Purpura (ATP)

A

Antibodies destroy platelets.

Type 2 autoimmunity.

15
Q

Autoimmune Hemolytic Anemia

A

Autoantigen to RBC antigen.

16
Q

Hashimoto Thyroiditis

A

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

17
Q

Aire

A

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.

18
Q

Dressler Syndrome

A

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

19
Q

Chronic Be Disease

A

Type 4. Chronic frustrated immune response.

20
Q

SCID

A

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

21
Q

Brutons

A

No B cell development.

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

22
Q

X linked Hyper IgM

A

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

23
Q

Common Variable Immunodefiency

A

Unknown cause.

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

24
Q

Rheumatic Heart Disease

A

Autoantibody to laminin on heart valves.

25
Q

Major Basic Protein

A

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

26
Q

Job Syndrome (hyperIgE syndrome)

A

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

27
Q

Selective IgA Deficiency

A

increased frequency and severity of allergies

28
Q

Ataxia Teangiectasia

A

autosomal recessive

29
Q

Wiskott-Aldrich Syndrome

A

X linked
platelet and B cell deficiency
eczema

30
Q

Ankylosing Spondylitis

A

HLA-B27

31
Q

Type 1 Diabetes

A

Antibodies made to islet associated antigens.

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

32
Q

Autoimmune Polyglandular Syndrome Type 1

A

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
33
Q

Chronic Granulomatous Disease (CGD)

A
  • 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