Immunodeficiency Diseases

Question 1

What is primary immunodeficiency?

Answer 1

A genetic defect that results in an increased susceptibility to infection. Frequently manifested in infancy and childhood

Question 2

What is secondary immunodeficiency?

Answer 2

A susceptibility to infection that developed as a consequence of malnutrition, disseminated cancer, treatment with immunosuppressive drugs, or infection of immune cells.

Question 3

Any loss of function mutation that affects a _____ has negative consequences for survival.

Answer 3

Toll-Like Receptor

Question 4

A defect in leukocyte maturation or activation will result in _____ immunodeficiency; whereas, a defect in effector mechanisms will result in ______ immunodeficiency.

Answer 4

Innate

Adaptive

Question 5

Immunodeficiencies may affect one or more components of the immune system including _____ and _______ of the adaptive immune system.

Answer 5

T lymphocytes

B lymphocytes

Question 6

Immunodeficiencies may affect one or more components of the immune system including _____ and _______ of the adaptive immune system.

Answer 6

T lymphocytes

B lymphocytes

Question 7

What is the principal consequence of an immunodeficiency?

Answer 7

Increased susceptibility to infection

Question 8

The types of ______ ______ are indicative of the type of immunodeficiency.

Answer 8

Recurring Infections

Question 9

Deficient ______ immunity usually results in increased susceptibility to infection by pyogenic bacteria.

Answer 9

humoral

Question 10

Boys with XLA have low/absent levels of _______ and display very small tonsils and lymph nodes.

Answer 10

B cells

Question 11

Patients with hyper-IgM immunodeficiency have swollen lymph nodes due to a defect in _______ ligand on helper T cells that signals B cells to go through isotype switching.

Answer 11

CD40

*without isotype switching there is an elevated level of IgM and very low concentrations of IgG, IgA, and IgE

Question 12

Deficient humoral immunity is usually treated via ________ and/or gamma-globulin therapy.

Answer 12

Prophylactic antibiotics

Question 13

Deficient humoral immunity is usually treated via ________ and/or gamma-globulin therapy.

Answer 13

Prophylactic antibiotics

Question 14

Deficient _______ immunity usually results in increased susceptibility to viruses and other intracellular pathogens.

Answer 14

Cell-mediated

Question 15

What is the difference in susceptibility between humoral and cell-mediated immunity?

Answer 15

Humoral = pyogenic bacteria
Cell-mediated = viruses and intracellular pathogens

Question 16

True or False: Treatment for humoral immunodeficiency is routine, but there are few treatments for deficient T cell response (cell-mediated).

Answer 16

True

Question 17

DiGeorge’s syndrome is not an immune specific disorder but rather a disease associated with the lack of _______ development.

Answer 17

Thymus

*B cell function is only impaired to the extent of needing helper T cells

Question 18

X-linked _____ is the most common form for combined immunodeficiency disease characterized by deficient T- and B-cell function.

Answer 18

SCID

Question 19

What are the two aims of current treatments for immunodeficiency disorders?

Answer 19

1. Minimize and control infections

2. Replace the defective or absent components of the immune system

Question 20

What are the two aims of current treatments for immunodeficiency disorders?

Answer 20

1. Minimize and control infections

2. Replace the defective or absent components of the immune system

Question 21

What is the current treatment of choice for various immunodeficiency diseases?

Answer 21

Bone Marrow Transplant