Deck 18: Immunodeficiency Disorders

Question 1

Primary immunodeficiencies

A)are inherited.
B)are severe.
C)alter the complement pathway.
D)affect a range of cell types.
E)are contagious.

Answer 1

A

Question 2

Primary immunodeficiencies are NOT

A)are the most common primary immunodeficiencies.
B)are generally not especially severe.
C)do not impair complement fixation.
D)both are the most common primary immunodeficiencies and are generally not especially severe.
E)both are the most common primary immunodeficiencies and do not impair complement fixation.

Answer 2

D

Question 3

Primary immunodeficiencies affecting T cells tend to be MORE severe than those affecting B cells because

A)T cells control the innate immune response.
B)T cells regulate antigen presentation.
C)T cells regulate the adaptive immune response.
D)T cells can trigger apoptosis.
E)None of the answers are correct.

Answer 3

C

Question 4

Primary immunodeficiencies that affect B cells are often characterized by

A)T cells help fully activate B cells.
B)T cells help trigger class switching.
C)T cells help regulate antigen presentation by dendritic cells.
D)T cells help to both fully activate B cells and trigger class switching.
E)T cells help to both fully activate B cells and regulate antigen presentation by dendritic cells.

Question 5

Severe combined immunodeficiency (SCID)

A)is a result of the absence of macrophages or B cells.
B)affects only part of the adaptive immune response.
C)can be caused by failure of TAP transporters.
D)can be caused by failure of V(D)J recombination.
E)All of the answers are correct.

Question 6

Severe combined immunodeficiency (SCID) can be caused by

A)failure of V(D)J recombination.
B)failure of cytokine receptors.
C)failure of T-cell receptor signaling.
D)failure of lymphoid progenitor survival.
E)All of the answers are correct.

Question 7

Children born with SCID often first become infected with fungi or viruses because

A)their T cells function well enough to handle bacterial infection.
B)maternal antibodies provide protection against bacteria for several months.
C)their macrophage function is not impaired, and it can contain bacterial pathogens.
D)complement lysis of bacteria, especially by the alternative pathway, is sufficient protection.
E)None of the answers are correct.

Question 8

Deficiency in the common ? chain of cytokine receptors is a particularly severe problem because

A)it is encoded on the X chromosome, so males are hemizygous.
B)it is used by several cytokine receptors.
C)it leads to defects in T cells, B cells, and NK cells.
D)All of the answers are correct.
E)None of the answers are correct.

Question 9

Adenosine deaminase deficiency leads to SCID, even though

A)cells have alternate pathways to use.
B)it primarily affects dendritic cells.
C)it is only used in adults.
D)the gene is not part of the immune system.
E)None of the answers are correct.

Question 10

Bare lymphocyte syndrome does NOT

A)produce a phenotype like SCID.
B)result from a defect in the TAP transporter.
C)lead to a lack of MHC class II.
D)result in a deficiency of cytotoxic T cells.
E)increase susceptibility to viral infections.

Question 11

Hyper IgM syndrome

A)is autosomal.
B)is a result of a helper T-cell defect.
C)includes normal affinity maturation.
D)has impaired cytotoxic T-cell responses.
E)None of the answers are correct.

Question 12

X-linked agammaglobulinemia (X-LA)

A)is more common in women than men.
B)has increased IgG levels.
C)has increased susceptibility to fungal infections.
D)can be treated with antiviral medication.
E)results from a B-cell signaling defect.

Question 13

A defect in NADPH is associated with which of the following disorders?

A)Job syndrome
B)Chronic granulomatous disease
C)Severe combined immunodeficiency
D)X-linked agammaglobulinemia
E)None of the answers are correct.

Question 14

Defects in which cell type are commonly associated with increased cancer risk?

A)NK cells
B)T cells
C)B cells
D)Macrophages
E)Granulocytes

Question 15

Which of the following is NOT a good candidate for immunodeficiency replacement therapy?

A)Immunoglobulin
B)IFN- ?
C)Hematopoietic stem cells
D)Defective genes
E)All of the answers are good candidates.

Question 16

Which primary immunodeficiency has been co-opted in mice to study a secondary immunodeficiency?

A)SCID
B)RAG knockout
C)RAG/IL-2 ? chain knockout
D)APECED
E)Bare-lymphocyte syndrome

Question 17

Secondary immunodeficiencies

A)can be brought on by multiple factors.
B)can be contagious.
C)can be inherited.
D)can be both brought on by multiple factors and contagious.
E)can be both brought on by multiple factors and inherited.

Question 18

Hypogammaglobulinemia differs from common variable immunodeficiency in that it

A)is not heritable.
B)is characterized by low levels of IgG.
C)can be treated by IV-gamma globulin.
D)is relatively mild.
E)All of the answers are correct.

Question 19

Agent-induced immunodeficiency does NOT

A)lead to an immunosuppressed state.
B)accompany transplant treatments.
C)come about after radiation treatment.
D)get passed on in gametes.
E)All of the answers are true.

Question 20

Which of the following is NOT an acquired immunodeficiency?

A)HIV
B)Malnutrition
C)Acquired hypogammaglobulinemia
D)SCID
E)Extremes of age

Question 21

HIV-1

A)is a retrovirus.
B)belongs to the lentivirus family.
C)integrates as a provirus.
D)All of the answers are correct.
E)None of the answers are correct.

Question 22

HIV-1 and HIV-2 are very similar EXCEPT that

A)HIV-2 progresses far more quickly.
B)HIV-2 is resident in high percentages in some populations.
C)HIV-1 is thought to have arisen from FIV, Feline Immunodeficiency Virus.
D)HIV-2 is more widespread.
E)All of the answers are true.

Question 23

HIV-1 is NOT

A)most often transmitted by sexual contact.
B)most often transmitted by blood products.
C)able to be transmitted from mother to children during birth.
D)treatable by antiviral drugs.
E)able to progress over the course of many years.

Question 24

Which of the following routes is thought NOT to be a major factor in HIV-1 early infection?

A)Transmission by Langerhans cells
B)Activation of local CD4⁺ cells
C)Transmission by macrophages
D)Transmission by dendritic cells
E)All of the answers are major factors.

Question 25

The HIV-1 pol protein encodes

A)a protease.
B)an integrase.
C)a reverse transcriptase.
D)All of the answers are correct.
E)None of the answers are correct.

Question 26

Because reverse transcriptase is an error-prone enzyme, it follows that

A)HIV-1 will have a high mutation rate.
B)drugs targeting HIV will affect only part of any population.
C)treatment of HIV will produce temporary results only.
D)All of the answers are correct.
E)None of the answers are correct.

Question 27

Because the gp120 protein on HIV-1 interacts with both CD4⁺ and CCR5, it is NOT reasonable to conclude that

A)CD4⁺ cells will not be infected.
B)CCR5 cells will not be infected.
C)CCR5 deficiency should confer HIV-1 resistance.
D)blocking CCR5 should provide some protection.
E)blocking CD4⁺ is a useful therapeutic approach.

Question 28

Multidrug treatment of HIV-1 is preferred over single-drug treatment because

A)the high mutation rate of HIV-1 means that many drugs are not likely to work against it.
B)multidrug treatment places a more challenging selective pressure on the virus; to survive requires multiple mutations.
C)protecting itself against one drug makes the virus less able to protect itself against the others.
D)the drugs work synergistically with each other.
E)one drug makes the virus more susceptible to the others.

Question 29

Which of the following does NOT improve prognosis after exposure or infection with HIV?

A)Antiviral therapy
B)Infection with HIV-2
C)A strong antibody response
D)A strong and varied CD8⁺ T-cell response
E)A deletion in the host gene for CCR5

Question 30

What is often the FIRST clue that an individual may have a primary immune deficiency? Explain why it takes time for the deficiency to manifest, despite being inherited and present at birth.

Question 31

Define SCID and list three of its MOST common causes.

Question 32

How can a variety of single-gene mutations all lead to the same condition of MSMD, or Mendelian inheritance of susceptibility to mycobacterial diseases? Explain.

Question 33

Some immunodeficiencies can also be the cause of autoimmune diseases.Explain how this paradox is possible.

Question 34

Explain how HIV enters a new host.Once inside, how is HIV then able to enter and replicate inside of host cells?

Question 35

Characterize the three clinical phases of untreated HIV infection, starting with initial infection.

Question 36

Name two different classes of drugs that can prevent HIV replication.How are there so many drugs that work against the virus? Explain.

Question 37

Developing a vaccine against HIV is challenging - explain why.Describe two failures and one potential success.

Question 38

Identify three nondrug conditions that can lead to a person NOT developing AIDS after being exposed to HIV.Explain.

Question 39

Explain the relationship between the HIV receptors CD4⁺, CCR5, and CXCR4.