Deck 8: Immunodeficiency-Diseases

Question 1

A generalized deficiency in the ability to mount delayed-type hypersensitivity (DTH)responses,as described in question 3,is called:

A)Clinical atopy
B)Clonal ignorance
C)Clinical anergy
D)Clonal deletion
E)Seroconversion

Answer 1

Clinical anergy

Question 2

Match each of the following descriptions in questions with the appropriate disease (A-G).
Defect in leukocyte adhesion-dependent functions,resulting from mutations in the gene encoding the CD18 integrin β chain,and leading to recurrent bacterial and fungal infections

A)X-linked agammaglobulinemia
B)X-linked hyper-IgM syndrome
C)X-linked lymphoproliferative syndrome
D)Class II MHC deficiency
E)Chronic granulomatous disease (CGD)
F)Leukocyte adhesion deficiency-1 (LAD-1)
G)Leukocyte adhesion deficiency-2 (LAD-2)

Answer 2

Leukocyte adhesion deficiency-1 (LAD-1)

Question 3

Match each of the following descriptions in questions with the appropriate disease (A-G).
Impaired CD4+ T cell development and activation,resulting in defective cell-mediated immunity and impaired T cell-dependent humoral immunity
H.

A)X-linked agammaglobulinemia
B)X-linked hyper-IgM syndrome
C)X-linked lymphoproliferative syndrome
D)Class II MHC deficiency
E)Chronic granulomatous disease (CGD)
F)Leukocyte adhesion deficiency-1 (LAD-1)
G)Leukocyte adhesion deficiency-2 (LAD-2)

Answer 3

Class II MHC deficiency

Question 4

All of the following may result in an acquired immunodeficiency EXCEPT:

A)Malnutrition
B)Treatment with corticosteroids
C)Disseminated cancer
D)Inherited defect in B cell maturation
E)Infection with human immunodeficiency virus (HIV)

Question 5

Match each of the following descriptions in questions with the appropriate disease (A-G).
Defect in lymphocyte activation due to mutations in the gene encoding the SAP adapter protein required for inhibition of signaling by the SLAM molecule.Lack of SAP results in uncontrolled B cell proliferation,hypogammaglobulinemia,and B cell lymphoma in the setting of Epstein-Barr virus (EBV)infection.

A)X-linked agammaglobulinemia
B)X-linked hyper-IgM syndrome
C)X-linked lymphoproliferative syndrome
D)Class II MHC deficiency
E)Chronic granulomatous disease (CGD)
F)Leukocyte adhesion deficiency-1 (LAD-1)
G)Leukocyte adhesion deficiency-2 (LAD-2)

Question 6

In which of the following conditions would you be LEAST likely to see a robust delayed-type hypersensitivity (DTH)response to a Candida antigen skin challenge?

A)X-linked agammaglobulinemia
B)DiGeorge syndrome
C)Selective IgA deficiency
D)Common variable immunodeficiency
E)Chronic granulomatous disease

Question 7

Which of the following is not a valid explanation for why patients cannot eradicate HIV infections?

A)HIV does not stimulate a CD8+ CTL response.
B)HIV mutation rate is high,leading to evasion of immune recognition.
C)A reservoir of latently infected cells invisible to the immune system is established rapidly after infection.
D)CD4+ T cells are destroyed,impairing the immune response to the virus.

Question 8

Match each of the following descriptions in questions with the appropriate disease (A-G).
Defect in B cell maturation at the pre-B cell stage caused by mutations in the gene encoding the B cell tyrosine kinase (Btk),resulting in undetectable levels of serum Ig

A)X-linked agammaglobulinemia
B)X-linked hyper-IgM syndrome
C)X-linked lymphoproliferative syndrome
D)Class II MHC deficiency
E)Chronic granulomatous disease (CGD)
F)Leukocyte adhesion deficiency-1 (LAD-1)
G)Leukocyte adhesion deficiency-2 (LAD-2)

Question 9

Match each of the following descriptions in questions with the appropriate disease (A-G).
Defect in phagocyte microbicidal activity as a result of defective production of reactive oxygen intermediates,most commonly from a mutation in the gene encoding cytochrome b558

A)X-linked agammaglobulinemia
B)X-linked hyper-IgM syndrome
C)X-linked lymphoproliferative syndrome
D)Class II MHC deficiency
E)Chronic granulomatous disease (CGD)
F)Leukocyte adhesion deficiency-1 (LAD-1)
G)Leukocyte adhesion deficiency-2 (LAD-2)

Question 10

Which one of the following is typically NOT associated with T cell immunodeficiencies?

A)Cancer
B)Viral infection
C)Autoimmunity
D)Infection with intracellular microbes
E)Infection with pyogenic bacteria

Question 11

The progression of HIV infection to AIDS is most directly due to which of the following?

A)A loss of CD4+ T cells to numbers insufficient to prevent infections
B)Reduction in serum IgG levels to levels insufficient to prevent infections
C)Increased amount of virus in the blood
D)Loss of weight
E)Reduction in CD8+ CTLs specific for HIV

Question 12

Which molecules on the T cell surface are directly involved in HIV infection?

A)CD4 and CD25
B)CCR5 and CD25
C)CD3 and CD4
D)CD8 and CCR5
E)CD4 and CCR5