Deck 8: Congenital and Acquired Immunodeficiencies

Question 1

Matching
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)Bare lymphocyte syndrome
E)Chronic granulomatous disease (CGD)
F)Leukocyte adhesion deficiency-1 (LAD-1)
G)Leukocyte adhesion deficiency-2 (LAD-2)

Answer 1

Leukocyte adhesion deficiency-1 (LAD-1)

Question 2

Matching
Match each of the following descriptions in questions with the appropriate disease (A-G).
Impaired CD4+ T cell development and activation due to lack of class II MHC expression, resulting in defective cell-mediated immunity and impaired T cell-dependent humoral immunity

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

Answer 2

Bare lymphocyte syndrome

Question 3

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

Answer 3

Infection with pyogenic bacteria

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

Matching
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)Bare lymphocyte syndrome
E)Chronic granulomatous disease (CGD)
F)Leukocyte adhesion deficiency-1 (LAD-1)
G)Leukocyte adhesion deficiency-2 (LAD-2)

Question 6

Matching
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)Bare lymphocyte syndrome
E)Chronic granulomatous disease (CGD)
F)Leukocyte adhesion deficiency-1 (LAD-1)
G)Leukocyte adhesion deficiency-2 (LAD-2)

Question 7

Match the descriptions in questions with the associated HIV gene (A-G).
Early gene product; required for elongation of viral transcripts

A)env
B)gag
C)nef
D)pol
E)rev
F)tat
G)vif

Question 8

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 9

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

Question 10

Match the descriptions in questions with the associated HIV gene (A-G).
Late gene product; encodes reverse transcriptase, integrase, protease

A)env
B)gag
C)nef
D)pol
E)rev
F)tat
G)vif

Question 11

Match the descriptions in questions with the associated HIV gene (A-G).
Late gene product; encodes coat proteins gp120 and gp41

A)env
B)gag
C)nef
D)pol
E)rev
F)tat
G)vif

Question 12

Matching
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)Bare lymphocyte syndrome
E)Chronic granulomatous disease (CGD)
F)Leukocyte adhesion deficiency-1 (LAD-1)
G)Leukocyte adhesion deficiency-2 (LAD-2)

Question 13

Match the descriptions in questions with the associated HIV gene (A-G).
Late gene product; encodes nucleocapsid core and matrix proteins

A)env
B)gag
C)nef
D)pol
E)rev
F)tat
G)vif