Lecture 19: Primary and Secondary Immune Deficiencies

Question 1

____ Immunodeficiencies: congenital defects resulting in impaired immune responses
A. Primary
B. Secondary

Answer 1

A. Primary

Question 2

____ Immunodeficiencies: induced by environmental factors (infectious disease, chemical, physical,
or physiological causes)
A. Primary
B. Secondary

Answer 2

B. Secondary

Question 3

True or False: Malnourishment, neoplasia, and medical interventions can cause secondary immunodeficiencies

Answer 3

True

Question 4

Individuals with phagocytic
deficiencies commonly have infections with ____ extracellular pathogens

Answer 4

opportunistic

Question 5

What are two quantitative phagocytic deficiencies? What are three qualitative phagocytic deficiencies?

Answer 5

Quantitative
1. Congenital Agranulocytosis
2. Radiation Induced Neutropenia

Qualitative
1. Leukocyte Adherence Deficiency
2. Lazy Leukocytes Syndrome
3. Chronic Granulomatous Disease

Question 6

Which quantitative phagocytic deficiency is caused by absence of mature peripheral neutrophils?

Answer 6

Congenital Agranulocytosis

Question 7

Which qualitative phagocytic deficiency is caused by defective B chain of CD18

Answer 7

Leukocyte Adherence Deficiency

• CTL/NK cells cannot adhere
• Th2/B cells cannot form conjugates

Question 8

Symptoms of LAD?

Answer 8

Inability to form pus

Question 9

What causes Lazy Leukocyte Syndrome?

Answer 9

Defect in neutrophil response to chemotacic signals or deficiency in C3a/C5a production

Question 10

Defective intracellular killing due to mutation in G6PD or cytochrome b can cause which qualitative phagocytic deficiency?

Answer 10

Chronic Granulomatous Disease

Question 11

Allogenic stem cells cure which two phagocytic deficiencies?

Answer 11

Congenital Agranulocytosis
Leukocyte Adherence Deficiency

Question 12

Severe, recurrent bacterial (capsule producing) infections suggest which humoral immune deficiency?

Answer 12

Bruton’s X linked agammaglobulinemia
- cause: decrease in passively acquired IgG

Question 13

Pus producing infections are seen in which qualitative humoral immune deficiency?

Answer 13

X Linked Hyper-IgM Syndrome
- these people have no or low IgG, IgE, and IgA

Question 14

Which qualitative humoral immune deficiency is caused by defects in cytokine receptor expression and Th2 cytokine production (IL-5)?

Answer 14

Common Variable Hypogammagloulinemia

Question 15

True or False: DiGeorge’s Syndrome (congenital thymic aplasia) is a secondary T cell deficiency

Answer 15

False - primary T cell deficiency

Question 16

Abnormal thymus development and hypoparathyroidism can be caused by which 1 T cell deficiency?

Answer 16

DiGeorge’s Syndrome

Question 17

Low T Cell CD3 and low to no DTH suggests

Answer 17

Digeorge’s

Question 18

True or False: AIDS is a secondary T cell deficiency characterized by decreased CD4 T+ cells and infections by opportunistic pathogens

Answer 18

True

Question 19

What three disorders are combined humoral/cell mediated immunodeficiencies?

Answer 19

1) Reticular Dysgenesis
2) Bare Lymphocyte Syndrome
3) SCID

Question 20

What are four complement deficiencies?

Answer 20

1) C3 Deficiency
2) Deficiency in early components of classical pathway
3) Deficiency in complement components that form the MAC (C5,6,7, or C8)

Question 21

What causes reticular dysgenesis?

Answer 21

Myeloid and lymphoid stem cells fail to differentiate during hematopoiesis (therefore: no B, T cells, or neutrophils)

Question 22

In Type 1 Bare Lymphocyte Syndrome, there is no ___ expression while in Type 2 there is no ___ expression

Answer 22

HLA 1
HLA 1/2

Question 23

Faulty antigen presentation leads to failure of activated T cells to develop, which affects adaptive immune response

Answer 23

Bare Lymphocyte Syndrome

Question 24

__ is caused by deficiency in production of T cell dependent Ab and CMI

Answer 24

SCID

Question 25

Failure to thrive, decreased T and B cells are symptoms of ___

Answer 25

SCID

Question 26

Recurrent infections with meningococci or gonococci suggests deficiency in which complement components?

Answer 26

C5-C9
- C9 is most common

Question 27

Recurrent infections by encapsulated bacteria and failure to clear IC suggests which complement deficiency?

Answer 27

C3

Question 28

Increase in IC complex disease, rheumatoid diseases, and encapsulated bacterial infections, as well as inability to make enough C3b or opsins suggest _____

Answer 28

deficiency in early components of classical pathway (C1a, C1r, C1s, C2, and C4)

Question 29

Defect in C1 Inhibitor and hereditary angioedema suggest ____

Answer 29

complement regulatory component deficiencies