Deck 16: Immunodeficiency Disease

Question 1

The disease SCID is characterized by which of the following?

A) diagnosed in infancy
B) short life span
C) no antibody production
D) all of the above

Answer 1

all of the above

Question 2

Which is true of selective IgA deficiency?

A) It is invariably fatal.
B) It is very rare.
C) It mainly occurs in older adults.
D) Patients may develop an anti-IgA antibody.

Answer 2

Patients may develop an anti-IgA antibody.

Question 3

A nitro-blue tetrazolium (NBT)test indicates little color in cells observed under a microscope.What disease does this most likely indicate?

A) chronic granulomatous disease
B) ataxia-telangiectasia
C) Wiskott-Aldrich syndrome
D) transient hypogammaglobulinemia

Answer 3

chronic granulomatous disease

Question 4

Which of the following is associated with DiGeorge syndrome?

A) small or missing thymus
B) missing B-cell precursors
C) inherited as X-linked recessive
D) M spike in the gamma region

Question 5

A 7-month-old boy is seen by a specialist because of hypocalcemia,viral pneumonia,a history significant for recurrent diarrhea,and oral candidiasis.An x-ray reveals the lack of a thymic shadow.This boy most likely has:

A) DiGeorge syndrome
B) Wiskott-Aldrich syndrome
C) ataxia telangiectasia
D) bare lymphocyte syndrome

Question 6

Which disease may be expected to show an IgM spike on an electrophoretic pattern?

A) hypogammaglobulinemia
B) multicystic kidney disease
C) Waldenström's macroglobulinemia
D) Wiskott-Aldrich syndrome

Question 7

A male baby who has suffered from persistent Candida fungal infections and several bouts of pneumonia all before the age of 6 months is found to have some developmental problems as well as low-set ears.Lab testing reveals an absence of T cells.What is the most likely diagnosis?

A) selective IgA deficiency
B) common variable immunodeficiency
C) Waldenström's macroglobulinemia
D) DiGeorge syndrome

Question 8

A 1-year-old boy is evaluated for a suspected immune deficiency.He has a history since birth of eczema,recurrent rashes,and diarrhea.(Normal values are given in parentheses.)His WBC count is 6.75/uL (5.0-10.8 × 10³ uL)with a normal differential.Platelet count is 35,000 (150,000-350,000/uL)and the platelets are small.His IgG is 750 mg/dL (600-1,500 mg/dL),IgM is 30 mg/dL (75-150 mg/d:),IgA is 475 mg/dL (50-125 mg/dL),and IgE is 750 mg/dL (10-50 mg/dL).The boy's blood is typed as O-positive,and he does not have anti-A or anti-B antibodies.Flow cytometry performed on his blood shows 11% (5-20%)CD19 and 50% (60-80%)CD3 cells with a normal ratio of CD4:CD8.T-cell function tests are abnormal.This boy most likely has:

A) Bruton's hypogammaglobulinemia
B) Wiskott-Aldrich syndrome
C) DiGeorge syndrome
D) ataxia telangiectasia

Question 9

Which of the following best describes common variable immunodeficiency disease?

A) Symptoms appear in infancy.
B) It is inherited as an X-linked recessive.
C) It is invariably fatal for the patient.
D) There is a deficiency of IgA and/or IgG.

Question 10

A 2-week-old girl is evaluated by a specialist because her physical appearance is abnormal.She has been hospitalized since birth.(Normal values are given in parentheses.)Her CD3 level is reported as 15% (60-80%),CD4 = 10% (30-65%),CD8 = 5% (10-40%),and CD19 = 84% (5-20%).Serum immunoglobulin levels are normal.Karyotyping reveals a deletion on chromosome 22.This girl has:

A) Bruton's hypogammaglobulinemia
B) Wiskott-Aldrich syndrome
C) DiGeorge syndrome
D) ataxia telangiectasia

Question 11

A patient with hereditary angioedema will present with swelling in the tissues and has which of the following deficiencies?

A) C5,C6,C7,C8
B) phagocytic cell function
C) mature B cells
D) C1 inhibitor

Question 12

A child suspected of having an inherited humoral immunodeficiency disease is given diphtheria/tetanus vaccine.Two weeks after the immunization,his level of antibody to the specific antigens is measured.Which result is expected for this patient if he does have this deficiency?

A) increased levels of specific antibody
B) no change in the level of specific antibody
C) an increase in IgG-specific antibody but not IgM-specific antibody
D) increased levels of nonspecific antibody

Question 13

A 9-month-old infant is seen by a physician due to a persistent skin infection.His white and red blood cell counts are normal.However,immunofixation electrophoresis indicates a low level of IgG.When the infant returns to the physician for a follow-up visit in a month,his IgG level has increased.What condition do these results indicate?

A) SCID
B) transient hypogammaglobulinemia
C) common variable immunodeficiency
D) DiGeorge anomaly

Question 14

Decreased CD3-positive lymphocytes and a lack of responsiveness to phytohemagglutinin (PHA)in the circulation would typically be associated with:

A) inflammation
B) Bruton's hypogammaglobulinemia
C) DiGeorge syndrome
D) chronic granulomatous disease

Question 15

A 6-month-old baby has suffered from recurrent bacterial infections.Flow cytometry results indicate a lack of B cells but presence of normally functioning T cells.Which is the most likely diagnosis?

A) SCID
B) common variable hypogammaglobulinemia
C) DiGeorge syndrome
D) Bruton's agammaglobulinemia

Question 16

A 9-month-old boy was suspected of having an immune deficiency because he had many recurrent infections with Streptococcus pneumoniae.Laboratory tests revealed a normal percentage of T cells and T-cell subsets but a lack of mature B cells and immunoglobulins.This boy most likely has:

A) Wiskott-Aldrich syndrome
B) DiGeorge syndrome
C) X-linked agammaglobulinemia
D) chronic granulomatous disease

Question 17

An autosomal recessive disease characterized by the presence of abnormally large granules primarily affecting the function of lysosomes is known as:

A) Chédiak-Higashi syndrome
B) X-linked lymphoproliferative disease
C) leukocyte adhesion deficiency
D) common variable immunodeficiency

Question 18

Which of the following diseases results in an acquired or secondary immunodeficiency?

A) HIV infection
B) Bruton's agammaglobulinemia
C) leukocyte adhesion deficiency
D) Wiskott-Aldrich syndrome

Question 19

Which of the following statements about severe combined immunodeficiency (SCID)are true?

A) may be associated with a signal transduction defect
B) is accompanied by hypergammaglobulinemia
C) patients can be vaccinated with live virus
D) occurs only in males