Exam 3 Set 2 2017

Question 1

Defects in neutrophil NADPH oxidase system produce:

A. Chronic granulomatous disease
B. Chediak-Higashi disease
C. Leukocyte adhesion deficiency
D. Hashimoto's disease
E. Streptococcal infection

Answer 1

A. Chronic granulomatous disease

Question 2

X-linked agammaglobulinemia results from a mutation in:

A. IFNg receptor
B. The CIITA promoter protein
C. An HLA gene
D. CD40L (CD154) 
E. A tyrosine kinase gene

Answer 2

E. A tyrosine kinase gene

Question 3

DiGeorge syndrome results from a defect in:

A. Purine nucleoside phosphorylase
B. WASP
C. Thymic development
D. DNA repair
E. CD3

Answer 3

C. Thymic development

Question 4

Mutations in the gamma chain of the receptors for IL-2, 4, 7, 9 & 15 lead to:

A. Reticular dysgenesis
B. Bare lymphocyte syndrome
C. Hyper-IgM syndrome
D. Severe combined immunodeficiency (SCID)
E. Build-up of toxic nucleotide metabolites

Answer 4

D. Severe combined immunodeficiency (SCID)

Question 5

Poor skin tests to a range of microbial antigens such as tuberculin and mumps indicate a deficiency of:

A. NK cells
B. T cells
C. B cells
D. Phagocytosis
E. Opsonization

Answer 5

B. T cells

Question 6

Primary immunodeficiency producing susceptibility to infection by viruses and molds is due to:

A. B-cell deficiency
B. T- cell deficiency
C. Phagocyte deficiency
D. Complement deficiency
E. Eosinophil deficiency

Answer 6

B. T cell deficiency

Question 7

Deletions in the T-cell CD154 (CD40L) gene produce:

A. The hyper-IgM syndrome
B. Congenital X-linked agammaglobulinemia
C. IgA deficiency
D. Wiskott-Aldrich Syndrome
E. Deficiency in cytotoxic T-cell activity

Answer 7

A. The hyper-IgM syndrome

Question 8

Which one of these is due to thymic aplasia?

A. Hereditary angioneurotic edema
B. DiGeorge syndrome
C. X-linked hyper IgM syndrome
D. Severe combined immunodeficiency
E. Bare lymphocyte syndrome (MHC class I)
F. X-linked agammaglobulinemia
G. Leukocyte adhesion deficiency
H. Chronic granulomatous disease

Answer 8

B. DiGeorge syndrome

Question 9

Which one of these is due to defective TAP?

A. Hereditary angioneurotic edema
B. DiGeorge syndrome
C. X-linked hyper IgM syndrome
D. Severe combined immunodeficiency
E. Bare lymphocyte syndrome (MHC class I)
F. X-linked agammaglobulinemia
G. Leukocyte adhesion deficiency
H. Chronic granulomatous disease

Answer 9

E. Bare lymphocyte syndrome (MHC class I)

Question 10

Which one of these is due to a defective RAG1 or RAG2?

A. Hereditary angioneurotic edema
B. DiGeorge syndrome
C. X-linked hyper IgM syndrome
D. Severe combined immunodeficiency
E. Bare lymphocyte syndrome (MHC class I)
F. X-linked agammaglobulinemia
G. Leukocyte adhesion deficiency
H. Chronic granulomatous disease

Answer 10

D. Severe combined immunodeficiency

Question 11

Which one of these is due to defective C1 inhibitor?

A. Hereditary angioneurotic edema
B. DiGeorge syndrome
C. X-linked hyper IgM syndrome
D. Severe combined immunodeficiency
E. Bare lymphocyte syndrome (MHC class I)
F. X-linked agammaglobulinemia
G. Leukocyte adhesion deficiency
H. Chronic granulomatous disease

Answer 11

A. Hereditary angioneurotic edema

Question 12

Which one of these is due to defective CD40 ligand?

A. Hereditary angioneurotic edema
B. DiGeorge syndrome
C. X-linked hyper IgM syndrome
D. Severe combined immunodeficiency
E. Bare lymphocyte syndrome (MHC class I)
F. X-linked agammaglobulinemia
G. Leukocyte adhesion deficiency
H. Chronic granulomatous disease

Answer 12

C. X-linked hyper IgM syndrome

Question 13

Which one of these is due to defective Btk tyrosine kinase?

A. Hereditary angioneurotic edema
B. DiGeorge syndrome
C. X-linked hyper IgM syndrome
D. Severe combined immunodeficiency
E. Bare lymphocyte syndrome (MHC class I)
F. X-linked agammaglobulinemia
G. Leukocyte adhesion deficiency
H. Chronic granulomatous disease

Answer 13

F. X-linked agammaglobulinemia

Question 14

Which one of these if due to defective CD18?

A. Hereditary angioneurotic edema
B. DiGeorge syndrome
C. X-linked hyper IgM syndrome
D. Severe combined immunodeficiency
E. Bare lymphocyte syndrome (MHC class I)
F. X-linked agammaglobulinemia
G. Leukocyte adhesion deficiency
H. Chronic granulomatous disease

Answer 14

G. Leukocyte adhesion deficiency

Question 15

Which one of these is due to defective NADPH oxidase?

A. Hereditary angioneurotic edema
B. DiGeorge syndrome
C. X-linked hyper IgM syndrome
D. Severe combined immunodeficiency
E. Bare lymphocyte syndrome (MHC class I)
F. X-linked agammaglobulinemia
G. Leukocyte adhesion deficiency
H. Chronic granulomatous disease

Answer 15

Chronic granulomatous disease

Question 16

Which one of the following statements regarding inherited immunodeficiency diseases is correct?

A. Affected individuals are less susceptible to infection
B. Mortality rates are reduced by the administration of antibodies to affected individuals
C. Most deficiency syndromes are caused by dominant gene defects
D. Women are more likely than men to inherit X-linked immunodeficiencies
E. Extracellular bacterial infections are common in deficiency syndromes with T-cell defects

Answer 16

B. Mortality rates are reduced by the administration of antibodies to affected individuals

Question 17

Which of the following deficiency syndromes affects T-cell but not B-cell function?

A. X-linked agammaglobulinemia
B. X-linked hyper IgM syndrome
C. X-linked lymphoproliferative syndrome
D. X-linked SCID
E. X-linked Wiskott-Aldrich syndrome

Answer 17

E. X-linked Wiskott-Aldrich syndrome

Question 18

Which immune deficiencies result in defective phagocytic processes causing chronic bacterial infections? (Select all that apply)

A. Chediak-Higashi syndrome
B. Wiskott-Aldrich syndrome
C. Myeloperoxidase deficiency
D. X-linked agammaglobulinemia (XLA)
E. Chronic granulomatous disease (CGD)

Answer 18

A. Chediak-Higashi syndrome
C. Myeloperoxidase deficiency
E. Chronic granulomatous disease (CGD)

Question 19

Which of the following participates in the T-cell cytoskeletal reorganization required for T-cell cytokine production and cell-mediated interactions?

A. Adenosine deaminase (ADA)
B. Purine nucleotide phosphorylase (PNP)
C. Wiskott-Aldrich syndrome protein (WASP)
D. Myeloperoxidase
E. Bruton's tyrosine kinase (Btk)

Answer 19

C. Wiskott-Aldrich syndrome protein (WASP)

Question 20

Chronic granulomatous disease (CGD), a condition resulting in chronic bacterial and fungal infections, is caused by one or more defects in _____________, comprising the ability of macrophages to ________.

A. CD18; produce cell adhesion molecules
B. NADPH oxidase; produce superoxide radical
C. CD40 ligand; produce GM-CSF
D. C5-C9; defend against Neisseria
E. C3; opsonize capsulated bacteria

Answer 20

B. NADPH oxidase; produce superoxide radical

Question 21

Which genetic defect results in the accumulation of toxic levels of nucleotide metabolites and loss of T-cell function?

A. NADPH oxidase
B. Glucose-6-phosphate dehydrogenase
C. Myeloperoxidase
D. SH2D1A
E. Adenosine deaminase (ADA)

Answer 21

E. Adenosine deaminase (ADA)

Question 22

Bare lymphocyte syndrome leading to a lack of HLA class II molecule expression is due to a defect in:

A. Transcriptional regulators of HLA class II loci
B. The sequence of the conserved X box of the HLA class II promoter
C. A TAP peptide transporter
D. RAG-1 or RAG-2
E. Thymic development

Answer 22

A. Transcriptional regulators of HLA class II loci

Question 23

When an individual receives a kidney transplant, the main concern will be to control the development of:

A. Graft-versus-host disease
B. Transplant rejection
C. Xenorecognition
D. Allergic reactions
E. Lymphoproliferative disorders

Answer 23

B. Transplant rejection

Question 24

Which of the following are correctly matched? (Select all that apply)

A. Allograft; same person
B. Autograft; to treat damage caused by autoimmune processes
C. Isograft; syngeneic 
D. Antithymocyte globulin; xenogeneic
E. Same species; allogenic

Answer 24

C. Isograft; syngeneic
D. Antithymocyte globulin; xenogeneic
E. Same species; allogeneic

Question 25

Blood transfusions mismatched for ABO and/or rhesus antigens are associated with _________. (Select all that apply) A. Type III hypersensitivity reactions B. Alloreactive immune responses C. Lysis of recipient red blood cells D. Laboratory errors in the cross-matching procedure E. Activation of host complement and destruction of donor cells

Answer 25

B. Alloreactive immune responses D. Laboratory errors in the cross-matching procedure E. Activation of host complement and destruction of donor cells

Question 26

Alloantibodies to blood-vessel endothelium on solid organ grafts _____________. A. Are specific for HLA class I and class II antigens B. Cause hyperacute rejection C. Cause acute rejection D. Target endothelium for attack by NK cells E. Are IgA and do not fix complement

Answer 26

B. Cause hyperacute rejection

Question 27

Which of the following is a permissible match between a blood donor and a recipient (donor: recipient)? (Select all that apply) ``` A. O-: AB+ B. O+: AB- C. AB+ : O- D. A- : A+ E. AB- : O+ ```

Answer 27

A. O- : AB+ | D. A- : A+

Question 28

If _______ occurs in an organ to be transplanted, endothelial activation, leukocyte infiltration, inflammatory cytokine production and complement activation may occur. ``` A. Mixed lymphocyte reaction B. The transfusion effect C. Kidney dialysis D. Ischemia E. Myeloablative therapy ```

Answer 28

D. Ischemia

Question 29

Which of the following are correctly matched? (Select all that apply) A. Hyperacute rejection: preexisting antibodies against cell-surface antigens B. Acute rejection; anti-HLA antibodies C. Chronic rejection; alloreactive T cell clones specific for HLA allotypes of donor D. Acute rejection; direct pathway of allorecognition E. Transfusion effect: indirect pathway of allorecognition

Answer 29

A. Hyperacute rejection: preexisting antibodies against cell-surface antigens D. Acute rejection; direct pathway of allorecognition E. Transfusion effect: indirect pathway of allorecognition

Question 30

Acute rejection of a kidney graft involves the activation of recipient T cells by _______ of _______ origin ``` A. Dendritic cells; recipient B. B cells; recipient C. Dendritic cells; donor D. Macrophages; recipient E. B cells; donor ```

Answer 30

C. Dendritic cells; donor

Question 31

Which effector mechanisms of rejection resemble those responsible for type IV hypersensitivity reactions? ``` A. Xenogeneic B. Acute C. Chronic D. Hyperacute E. Blood transfusion ```

Answer 31

B. Acute

Question 32

What will happen if donor MHC donor self-peptide complexes activate recipient T cells? A. Acute rejection of transplanted organs occurs B. Suppression occurs and transplanted organs are tolerated C. Hyperacute rejection of transplanted organs occurs D. Complement pathways are activated E. An indirect pathway of allorecognition occurs

Answer 32

A. Acute rejection of transplanted organs occurs

Question 33

In a mixed lymphocyte reaction the donor cells are irradiated to ensure that they do not ________ ``` A. Stimulate recipient cells B. Become anergic C. Alter their level of expression of HLA molecules D. Proliferate E. Undergo apoptosis ```

Answer 33

D. Proliferate

Question 34

As time progresses following an organ transplant, the alloreactive T cell response shifts from a(n) ________ pathway to a(n) _________ pathway of allorecognition ``` A. Exogenous; endogenous B. Inflammatory; ctyotoxic C. Hyperacute; suppressive D. Autologous; heterologous E. Direct; indirect ```

Answer 34

E. Direct; indirect

Question 35

Alloantibody production after organ transplantation involves ___________ A. A mixed lymphocyte reaction B. The indirect pathway of allorecognition by CD4 T cells C. Activation of regulatory CD4 T cells D. The transfusion effect E. A switch from a chronic to an acute state of organ rejection

Answer 35

B. The indirect pathway of allorecognition by CD4 T cells

Question 36

In chronic rejection, effector T cells respond to _______ complexes on __________ -derived dendritic cells ``` A. Donor MHC class I: donor self peptide; donor B. Donor MHC class II: donor self peptide; donor C. Recipient MHC class I: donor MHC peptide; recipient D. Recipient MHC class II: donor MHC peptide; recipient E. Recipient MHC class II: donor MHC peptide; donor ```

Answer 36

D. Recipient MHC class II: donor MHC peptide; recipient

Question 37

The extent to which an individual's T cells respond to allogeneic HLA expressed on irradiated donor cells can be measured in vitro using ``` A. A cross-match test B. A superantigen recognition test C. The mixed lymphocyte reaction D. The transfusion effect assay E. The panel reactive antibody test ```

Answer 37

C. The mixed lymphocyte reaction