Primary Innate Immunodeficiency

Question 1

How are Primary Immunodeficiencies acquired?

Answer 1

Genetics

Question 2

What are the Primary Innate Immunodeficiencies - leukocyte disorders?

Answer 2

Leukocyte Adhesion Deficiency 1 and 2
Chediak Higashi Syndrome
Chronic Granulomatous Disease

Question 3

What are the Primary Innate Immunodeficiencies - leukocyte disorders?

Answer 3

Leukocyte Adhesion Deficiency 1 and 2
Chediak Higashi Syndrome
Chronic Granulomatous Disease

Question 4

Leukocyte disorders cause an issue in what cell type mediated immune response?

Answer 4

Neutrophils

Question 5

Leukocyte Adhesion Deficiency 1 has a defective?

Answer 5

Beta2 Integrin

Question 6

Defective Beta2 integrin

Answer 6

Leukocyte Adhesion Deficiency 1

Question 7

Leukocyte Adhesion Deficiency 2 has a lack of?

Answer 7

Selectin ligands (NO fucosyl transferase)

Question 8

Lack of selectin ligands (no fucosyl transferase)

Answer 8

Leukocyte Adhesion Deficiency 2

Question 9

Leukocyte Adhesion Deficiencies and what they lack?

Answer 9

LAD 1 - Defective Beta2 Integrin

LAD 2 - Lack selectin ligands (no fucosyl transferase)

Question 10

How is Chediak Higashi Syndrome inherited?

Answer 10

Autosomal Recessive

Question 11

Chediak Higashi Syndrome has a failure of?

Answer 11

Failure of phagolysosomal fusion

Question 12

Failure of phagolysosomal fusion

Answer 12

Chediak Higashi Syndrome

Question 13

Symptoms of Chediak Higashi Syndrome?

Answer 13

Albinism - grey hair

Easy bruising and bleeding

Question 14

Albinism with grey hair and easy bruising is what primary innate immunodeficiency?

Answer 14

Chediak Higashi Syndrome

- failure of phagolysosomal fusion

Question 15

What will be seen morphologically with Chediak Higashi Syndrome?

Answer 15

Giant granules

- Failure of phagolysosomal fusion

Question 16

Chronic Granulomatous Disease has failure of?

Answer 16

Failure of superoxide production within phagocytes

Question 17

Failure of superoxide production within phagocytes

Answer 17

Chronic Granulomatous Disease

Question 18

Chronic Granulomatous Disease involves no superoxide production in phagocytes. What happens?

Answer 18

Macrophages “wall off” infections

Question 19

Macrophages “walling off” infections is seen with?

Answer 19

Chronic Granulomatous Disease

Question 20

Leukocyte disorders of primary immunodeficiency are at risk for what type of infections?

Answer 20

Bacterial - s. aureus, strep/enterococcus

Question 21

Primary innate immunodeficiency complement disorder?

Answer 21

Membrane Attack Complex Deficiency

Question 22

Primary innate immunodeficiency complement disorder?

Answer 22

Membrane Attack Complex Deficiency

Question 23

What parts of the MAC complex can be affected with the primary disorder?

Answer 23

C5 - C9

Question 24

If there is no MAC, what will no occur?

Answer 24

NO lysis of microbe

Question 25

Membrane Attack Complex Deficiencies are at risk for what type of infections?

Answer 25

Neisseria

– meningitis

Question 26

Is Hereditary Angeioedema an immunodeficiency?

Answer 26

NOOO

Question 27

How is Hereditary Angioedema inherited?

Answer 27

Autosomal Dominant

Question 28

What is deficient with Hereditary Angioedema?

Answer 28

C1 - inhibitor

Question 29

What is unregulated with Hereditary Angioedema?

Answer 29

Kallikrein and complement pathways are unregulated

Question 30

Symptoms of Hereditary Angioedema?

Answer 30

Edema at multiple sites