Defenses and Deficiencies Part 4

Question 1

If a patient has a deficiency in TLR 4 receptors, what are they in danger of?

Answer 1

Infection by gram negative cells.

Question 2

What are the 6 steps of diapedisis?

Answer 2

1. Macrophage phagocytoses a bacteria/ Mast cell degranulation
2. Cytokine production from macrophage.
3. Macrophage upregulate cell adhesion molecules (selectins) and integrins.
4. Leukocytes bind to endothelial wall.
5. Leukocyte spreads out on endothelial wall
6. Leukocyte squeezes between endothelial cells.

Question 3

What are in the primary granules of neutrophils?

Answer 3

Myeloperoxidases(H202,OH-,Cl- into bleach), proteases, lipases nucleases.

Question 4

What is the difference between neutrophils and macrophages?

Answer 4

Neutrophils die along with the bacteria from the bleach that they make; This forms pus.

Question 5

What is pus?

Answer 5

Dead pathogens and dead neutrophils.

Question 6

How are eosinophils like neutrophils?

Answer 6

They can perform diapedisis and phagocytose pathogens.

Question 7

Which cytokine can recruit an eosinophil? Who produces it?

Answer 7

IL-4; Produced by Mast cells.

Question 8

What is the role of the eosinophil?

Answer 8

Play a role in asthma, and the destruction of helminths.

Question 9

How are eosinophil activation similar to mast cell activation?

Answer 9

They are activated the same way Mast cells are activated; Causes degranulation.

Question 10

How is eosinophil activation different to mast cells?

Answer 10

In addition to IgE receptors, they have IL-5 receptors that also need to be engaged before granules are released.

Question 11

What are the contents of the eosinophil granules?

Answer 11

Major Basic Protein, Acid phosphatase, Elastase, Eosinophil peroxidase, and eosinophilic cationic proteins.

Question 12

What does Major Basic Protein do?

Answer 12

Induces mast cell degranulation and digests sulfated proteoglycans, parasite cutilcles (their shell).

Question 13

What does Acid phosphatase and elastase do?

Answer 13

Degrade tissues like epidermis of helminthes, yeast and fungi

Question 14

What does eosinophil peroxidase do?

Answer 14

Catalyses respiratory burst in eosinophils.

Question 15

What does Eosinophilic cationic protein do?

Answer 15

Digests RNA and ribosomes; Anti-viral

Question 16

What can eosinophils do that mast cells cant do?

Answer 16

Phagocytose and release antihistamines; Negative feedback for mast cells.

Question 17

What is Septic shock?

Answer 17

The end result of sepsis; Widespread activation of mast cells and macrophages through TLRs. Acute response to bacteremia.

Question 18

How do you recognize septic shock?

Answer 18

High serum levels of TNF-a and IL-1; Systemic mast cell degranulation, Vascular permeability, HYPOTENSION (shock)

Question 19

What is the effect of septic shock on body temp and WBC numbers?

Answer 19

Body temperature rises and more WBCs are created.

Question 20

What are some immunodeficiencies of the innate immune system?

Answer 20

Chronic granulomatosis disease, Leukocyte adhesion deficiency 1&2, Chediak-Higashi sydrome, and complement deficiencies.

Question 21

Describe chronic granulomatosis disease.

Answer 21

Defective production of ROS by phagocytes; Mutation in phagocyte oxidate. Pathogen is PROTECTED by macrophages that are unable to do their job. Causes granulomas.

Question 22

Describe LAD1.

Answer 22

Absent or deficient expression of Beta 2 integrins, causing defective leukocyte adhesion.

Question 23

Describe LAD2

Answer 23

Absent or deficient expression of leukocyte ligands for endothelial E&P selectins; Failure of leukocyte migration out of vessel.

Question 24

Describe Chediak-Higashi syndrome.

Answer 24

Defective lysosomal function due to premature fusion during development in neutrophils, macrophages and dendritic cells. Defective granule function in NK cells and CD8. Partial albinism, defective platelets. Phagocytosis is not affected. Autosomal recessive.

Question 25

Describe Sporadic Congenital Neutropenia.

Answer 25

Due to mutations in the genes involved in granulocytic hematopoiesis and cell maturation; Severe arrest of granulopoiesis at promyelocyte stage. Neutrophil counts are low with severe onset of bacterial infections in infancy.

Question 26

Describe Lazy Leukocyte Syndrome.

Answer 26

Slow moving leukocytes as a result of defective cytoskeletal protein polymerization; Presents with constant bacterial infections of skin, mucous membranes, and urinary tract.

Question 27

What is the major characteristic of LAD?

Answer 27

There is LITTLE TO NO PUS in infected tissues.

Question 28

What is the common treatment for these innate immune deficiencies?

Answer 28

Bone marrow transplant