Cellular and Molecular Immunology 8th

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Abbas: Cellular and Molecular Immunology, 8th Edition
Congenital and Acquired Immunodeficiencies

Test Bank

Multiple Choice

1. 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)

ANS: D. An inherited defect in B cell maturation is, by definition, a primary

immunodeficiency, not an acquired immunodeficiency. In particular, abnormalities in B
lymphocyte development and function result in deficient antibody production and
increased susceptibility to infection by extracellular microbes. In contrast, malnutrition,
treatment with corticosteroids, disseminated cancer, and infection with human
immunodeficiency virus (HIV) would all result in acquired, or secondary,
immunodeficiencies. Administration of corticosteroids to intentionally create an
immunosuppressive state, such as in the treatment of inflammatory diseases or the
prevention of tissue allograft rejection, is referred to as iatrogenic immunosuppression.

2. 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

ANS: E. B cell, not T cell, immunodeficiencies are associated with increased incidence of
infection with pyogenic bacteria. In contrast, increased incidence of cancer is most often
seen in T cell immunodeficiencies, because T cells are critical for surveillance against
oncogenic viruses and the tumors they cause. Defects in cell-mediated immunity lead to
infection by viruses and other intracellular microbes. Interestingly, certain
immunodeficiencies are associated with increased incidence of autoimmunity; the
mechanism for this phenomenon may reflect a deficiency of regulatory T cells that
normally serve to maintain self-tolerance.

Questions 3 and 4
3. 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

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D. Common variable immunodeficiency

E. Chronic granulomatous disease

ANS: B. A delayed-type hypersensitivity (DTH) response is an immune reaction in

which T cell–dependent macrophage activation and inflammation cause tissue injury. The
severity of the reaction to the subcutaneous injection of antigen is used as an assay for
cell-mediated immunity. A deficient cutaneous DTH response to common microbial
antigens, such as Candida antigens, suggests a T cell immunodeficiency. DiGeorge
syndrome is a selective T cell deficiency caused by a congenital malformation that results
in defective development of the thymus and other embryologically associated structures.
In contrast, X-linked agammaglobulinemia, selective IgA deficiency, and common
variable immunodeficiency are B cell immunodeficiencies. Chronic granulomatous
disease is a congenital disorder in which phagocytes cannot generate free radicals and kill
ingested microbes.

4. 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

ANS: C. A generalized deficiency in delayed-type hypersensitivity (DTH) responses is

called clinical anergy. Note that clonal (or lymphocyte) anergy refers to the failure of
clones of T or B cells to react to antigen. Anergy may be a mechanism of maintaining
immunologic tolerance to self. Atopy refers to allergic disease. Clonal ignorance and
clonal deletion are mechanisms of tolerance to antigens. Seroconversion refers to the
appearance in the serum of antibodies specific for a particular antigen after infection or
immunization.

Matching

Questions 5-9
Match each of the following descriptions in questions 5-9 with the appropriate disease
(A-G).
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)

5. 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

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Test Bank 3

ANS: D. Bare lymphocyte syndrome is a form of severe combined immunodeficiency

disease (SCID) caused by inherited mutations in genes encoding factors required for class
II MHC transcription. The absence of class II MHC in the thymus results in a lack of
positive selection and maturation of CD4+ T cells.

6. 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

ANS: F. LAD-1 is a rare disorder in which integrins with the CD18 chain, such as LFA-
1, are not expressed. Migration and function of neutrophils, monocytes, and lymphocytes
are all impaired.

7. 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.

ANS: C. X-linked lymphoproliferative syndrome is characterized by a severe CD8 + T

cell response to Epstein-Barr virus (EBV), the virus that causes infectious
mononucleosis. In a way, this is an example of a lethal exaggerated response to an
infection, rather than a true immunodeficiency.

8. 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

ANS: A. Btk is required for pre-B cell receptor signaling and maturation from pre-B to
immature B cells. In females carrying the mutated Btk gene on one X chromosome, the B
cells that do mature have all inactivated the X chromosome carrying the mutant allele;
thus, these individuals do not show a clinical phenotype. This disease is effectively
controlled by injections of pooled gamma globulin.

9. 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

ANS: E. Chronic granulomatous disease (CGD) is a disorder of the innate immune

system. The failure to generate reactive oxygen intermediates (free radicals) leads to
persistent intracellular bacterial infections, with chronic T cell stimulation of
macrophages and development of granulomas.

Questions 10-13
Match the descriptions in questions 10-13 with the associated HIV gene (A-G).
A. env
B. gag
C. nef

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D. pol
E. rev
F. tat
G. vif

10. Late gene product; encodes reverse transcriptase, integrase, protease

ANS: D. The pol (polymerase) gene encodes the enzymes required for viral genome
integration into the host cell genome (reverse transcriptase, integrase, protease,
ribonuclease). Integrase is critical for processing viral proteins.

11. Early gene product; required for elongation of viral transcripts

ANS: F. tat is essential for HIV gene expression and serves to enhance the formation of
full length RNA transcripts from the proviral DNA.

12. Late gene product; encodes coat proteins gp120 and gp41

ANS: A. The env gene encodes a precursor polypeptide that is cleaved to form the gp120
and gp41 glycoproteins found in the HIV envelope; gp120 and gp41 are involved in CD4
binding and membrane fusion, respectively.

13. Late gene product; encodes nucleocapsid core and matrix proteins

ANS: B. The gag gene encodes the p24 (capsid) and p17 (matrix) proteins required for
packaging the human immunodeficiency virus (HIV) genome. Antibodies against these
proteins are used as diagnostic markers of HIV infection.

Copyright © 2015 by Saunders, an imprint of Elsevier Inc.