EX2 Immunodeficiency Diseases - Powell

Question 1

This type of immunodeficiency is caused by genetic defects that result in an increased susceptibility to infection

Answer 1

primary or congenital immunodeficiency

Question 2

What are the four main factors that can lead to secondary or acquired immunodeficiency

Answer 2

malnutrition
disseminated cancer
immunosuppressive drugs
infection of immune system cells

Question 3

Which type of immunodeficiency are frequently manifested in children and affect about 1/500 people in the USA

Answer 3

primary or congenital immunodeficiency

Question 4

This is vital for the defense against infectious organisms and their toxic products

Answer 4

the integrity of the immune system

Question 5

These are conserved across widely diverse species and any loss-of-function mutation has negative consequences for survival

Answer 5

Toll-like receptors

Question 6

True or False

primary immunodeficiencies may affect one or more components of the immune system (T-cells, B-cells, NKs, etc.)

Answer 6

True

Question 7

With primary immunodeficiency, it may result from what defects

Answer 7

defects in leukocyte maturation or activation or from defects in effector mechanisms of innate and adaptive immunity

Question 8

What is the principal consequence of an immunodeficiency disease

Answer 8

increased susceptibility to infection

Question 9

The nature of the infection in a particular patients depends largely on what

Answer 9

the component of the immune system that is defective

Question 10

A deficient humoral immunity will result in what

Answer 10

increased susceptibility to infection by pyogenic bacteria

Question 11

With X-linked Agammaglobulinemia, this is very low, these are usually absent, and these are present but in reduced numbers

Answer 11

all antibody isotopes are very low
circulating B cells are usually absent
pro-B cells are present but reduced numbers in bone marrow

Question 12

What physical attribution are associated with X-linked agammaglobulinemia

Answer 12

tonsils are very small
lymph nodes are barely palpable
thymus is normal (as are other T cell centers)

Question 13

X-linked agammaglobulinemia’s defect is associated with

Answer 13

loss of function of the Bruton Tyrosine Kinase that is important for pre-B cell expansion and maturation into Ig-expresion B cells

Question 14

Deficient cell-mediated immunity will result in what

Answer 14

increased susceptibility to viruses and other intracellular pathogens

Question 15

True or False

There are many different treatments for defects associated with deficient T cell responses

Answer 15

False; there are few, if any treatments

Question 16

True or False

It is rare that patients with absolute defects in T-cell function survive beyond infancy or childhood

Answer 16

True

Question 17

DiGeorge’s syndrome is a developmentally-related disease where what fails to develop

Answer 17

the thymus

Question 18

What is increased in DiGeorge’s syndrome, and also impaired

Answer 18

the percentage of B cells increases, but the function is impaired only to the extent of needing helper T cells

Question 19

What is the outcome of patients with DiGeorge’s syndrome

Answer 19

most infants die from infections, and those who make it past infancy usually develop some kind of mental retardation

Question 20

True or False

Severe combined immunodeficiency (T cell and B cell deficiencies) are rare and fatal

Answer 20

True

Question 21

True or False
X-linked SCID (XSCID) is the most common x-linked recessive severe combined immunodeficiency disease, accounting for approximately 42% of cases

Answer 21

True

Question 22

X-linked SCID patients develop what and lack this

Answer 22

develop persistent infection with opportunistic organisms

lack the ability to reject foreign tissues; at risk for GVHD

Question 23

X-linked SCID patients have few or no what (2), but an elevated percentage of this

Answer 23

T cells and NK cells
B cells (but they do not produce Ig)

Question 24

What are the aims of immunodeficiency treatments

Answer 24

to minimize and control infections

to replace the defective or absent components of the immune system by adoptive transfer/transplantation

Question 25

This is a valuable treatment for agammaglobulinemic patients, and has been life saving

Answer 25

passing immunization with pooled gamma globulin

Question 26

This is the current treatment of choice for various immunodeficiency diseases; and has been successful for SCID and similar diseases

Answer 26

bone-marrow transplantation

Question 27

The course of HIV after the primary infection and immune response is what

Answer 27

clinical latency (establishment of chronic infection, low-level viral production)

Question 28

After HIV is deem clinically latent, what then happens after other microbial infections

Answer 28

there is increased viral replication

Question 29

After HIV undergoes rapid replication, what occurs

Answer 29

AIDS; destruction of lymphoid tissues, depletion of CD4 T cells, and death

Question 30

True or False

HIV doesn’t cause immunodeficiency, it causes AIDS which does

Answer 30

True