CHAPTER 19: Immunodeficiency Diseases

Question 1

Patients with which immunodeficiency syndrome should receive irradiated blood products to protect against the development of GVHD?
a. Bruton’s thymidine kinase (Btk) deficiency
b. Selective IgA deficiency
c. SCID
d. CGD

Answer 1

c. SCID

Question 2

T-cell subset enumeration by flow cytometry would be most useful in making the diagnosis of which disorder?
a. Bruton’s thymidine kinase (Btk) deficiency
b. Selective IgA deficiency
c. SCID
d. Multiple myeloma

Answer 2

c. SCID

Question 3

What clinical manifestation would be seen in a patient with myeloperoxidase deficiency?
a. Defective T-cell function
b. Inability to produce IgG
c. Defective NK cell function
d. Defective neutrophil function

Answer 3

d. Defective neutrophil function

Question 4

Defects in which branch of the immune system are most commonly associated with severe illness after administration of live virus vaccines?
a. Cell-mediated immunity
b. Humoral immunity
c. Complement
d. Phagocytic cells

Answer 4

a. Cell-mediated immunity

Question 5

Which of the following statements applies to Bruton’s thymidine kinase (Btk) deficiency?
a. It typically appears in females.
b. There is a lack of circulating CD19+ B cells.
c. T cells are abnormal.
d. There is a lack of pre-B cells in the bone marrow.

Answer 5

b. There is a lack of circulating CD19+ B cells.

Question 6

DiGeorge anomaly may be characterized by all of the following except
a. autosomal recessive inheritance.
b. cardiac abnormalities.
c. parathyroid hypoplasia.
d. decreased number of mature T cells.

Answer 6

a. autosomal recessive inheritance.

Question 7

A 3-year-old boy is hospitalized because of recurrent bouts of pneumonia. Laboratory tests are run and the following findings are noted: prolonged bleeding time, decreased platelet count, increased level of serum alpha-fetoprotein, and a deficiency of naturally occurring isohemagglutinins. Based on these results, which is the most likely diagnosis?
a. PNP deficiency
b. Selective IgA deficiency
c. SCID
d. Wiskott-Aldrich syndrome

Answer 7

d. Wiskott-Aldrich syndrome

Question 8

Which of the following is (are) associated with ataxia-telangiectasia?
a. Inherited as an autosomal recessive
b. Defect in both cellular and humoral immunity
c. Chromosomal breaks in lymphocytes
d. All of the above

Answer 8

d. All of the above

Question 9

A 4-year-old boy presents with recurrent wound and soft-tissue infections. Which of the following assays should be considered for diagnosing his presumed PID?
a. DHR reduction
b. CD4 quantitation
c. CD19 quantitation
d. CD56 quantitation

Answer 9

a. DHR reduction

Question 10

A patient with a deficiency in complement component C7 would likely present with
a. recurrent Staphylococcal infections.
b. recurrent Neisserial infections.
c. recurrent E coli infections.
d. recurrent Nocardia infections.

Answer 10

b. recurrent Neisserial infections.

Question 11

A FoxP3 gene mutation may lead to a deficiency of what cell type?
a. T helper cells
b. T cytotoxic cells
c. B cells
d. T regulatory cells

Answer 11

d. T regulatory cells

Question 12

The Cylex ImmunoKnow assay is useful in determining functional capabilities of which cell type?
a. T cells
b. B cells
c. NK cells
d. Neutrophils

Answer 12

a. T cells

Question 13

Recurrent, periodic fevers may be associated with increased production of which immunoglobulin?
a. IgG
b. IgM
c. IgD
d. IgE

Answer 13

c. IgD

Question 14

Chronic mucocutaneous candidiasis, a PID that was previously thought to be a cell-mediated deficiency, is now classified as which type of PID?
a. Autoinflammatory disorder
b. Complement deficiency
c. Predominantly antibody deficiency
d. Innate immunity deficiency

Answer 14

d. Innate immunity deficiency

Question 15

Prenatal screening for SCID involves detecting
a. Tregs.
b. TRECS.
c. THELPS.
d. TCYTOS.

Answer 15

b. TRECS.