Deck 15: Primary Immunodeficiency Diseases

Question 1

To diagnose an immunodeficiency of
a.B cells, serum protein electrophoresis and immunoglobulin quantitation by nephelometry is performed
b.T cells, live yeast is injected intradermally, and the size of the delayed type of hypersensitivity reaction is measured
c.T and B cells, a spleen biopsy is performed followed by immunohistochemistry
d.macrophage, the enzyme levels are measured

Answer 1

B cells, serum protein electrophoresis and immunoglobulin quantitation by nephelometry is performed

Question 2

Several different defects can cause which of the following?
a.common variable immunodeficiency
b.SCID
c.selective IgA deficiency
d.all of the above

Answer 2

all of the above

Question 3

Lack of tonsils as an indicator of the lack of B cells is seen in baby boys with which of the following immunodeficiencies/
a.DiGeorge syndrome
b.Bruton's disease
c.Purine neucleoside phosphorylase
d.LAD

Answer 3

Bruton's disease

Question 4

(1)_________ infections suggest defects in antibody production, (2)________ infections suggest defects in T cell function, and both types of infections suggest(3) _________________
a.(1) fungal, (2) viral, (3) SCID
b.(1) viral, (2) fungal, (3) neutrophil defects
c.(1) bacterial, (2) viral, (3) SCID
d.(1) bacterial (2) viral, (3) DiGeorge,

Question 5

Purine nucleoside phosphorylase deficiency can cause
a.a buildup of excessive deoxy-GTP
b.the buildup of deoxyadenosine and S-adenosylhomocysteine
c.mutations in the IL-2 receptor
d.a defect in glycosylation of immunoglobulin

Question 6

Which of the following patients have no tonsils? Patients with
a.DiGeorge syndrome
b.Common variable immunodeficiency
c.Bruton's agammaglobulinemia
d.Transient hypogammaglobulinemia of infancy

Question 7

In this type of immunodeficiency, patients initially make their own immunoglobulin and then they develop
a.Bruton's hypogammaglobulinemia
b.DiGeorge syndrome
c.common variable hypogammaglobulinemia
d.Stigel-Keil hypogammaglobulinemia

Question 8

Chronic granulomatous disease is a caused by
a.defect in a subunit of NADPH oxidase that results in decreased ability to kill bacteria in the phagolysosome
b.defect in a subunit of nitroblue-tetrazolium that results in decreased ability to kill bacteria in the phagolysosome
c.defect in a subunit of NADPH oxidase that results in decreased ability to perform chemotaxis
d.defect in a subunit of nitroblue-tetrazolium that results in decreased ability to perform chemotaxis

Question 9

Hereditary angioedema is due to a defect in
a.phagocytic cells
b.C1-INH
c.C5-9
d.peroxidase

Question 10

IgA deficiency is related to
a.early demise as the result of infection
b.increased viral and fungal infections
c.allergic reactions to plasma during surgery
d.A and B

Question 11

Treatment of DiGeorge syndrome can include
a.passive transfer of immunoglobulin
b.bone marrow transplantation
c.transplantation of a fetal thymus
d.any of the above

Question 12

In which of the following types of immunodeficiency do patients initially not make their own immunoglobulin and then they do
a.Bruton's hypogammaglobulinemia
b.DiGeorge syndrome
c.common variable hypogammaglobulinemia
d.Stigel-Keil hypogammaglobulinemia
e.transient hypogammaglobulinemia

Question 13

Infections that suggest defects in antibody production are
a.fungal
b.viral
c.bacterial
d.all of these

Question 14

In DiGeorge syndrome,
a.some patients have complete lack of T cells
b.all patients have a complete lack of T cells
c.some patients have complete lack of B cells
d.all patients have a complete lack of B cells

Question 15

SCID is
a.always caused by Adenosine deaminase deficiency and results in no B or T cell response
b.always caused by reticular dysgenesis and results in no B or T cell response
c.a term used to describe a variety of different conditions that results in no B or T cell response
d.caused by a missing piece (a small deletion) of chromosome 22 that results in no B or T cell response

Question 16

Treatment of IgG hypogammaglobulinemia involves
a.a bone marrow transplant and infection management
b.passive transfer of immune oglobulin and infection management
c.plasmapheresis and infection management
d.antibiotic use and chemotherapy

Question 17

Purine nucleoside phosphorylase deficiency results in
a.decreased T-cell numbers
b.decreased B-cell numbers
c.decreased phagocytosis
d.increased autoimmunity

Question 18

To measure T-cell function, we perfor0m
a.serum protein electrophoresis
b.intradermal injection of tetanus toxoid
c.the NBT test
d.CD18 surface marker expression

Question 19

The first gene therapy was for a patient with which of the following disorders?
a.adenosine deaminase deficiency
b.common variable immunodeficiency
c.Bruton's agammaglobulinemia
d.transient hypogammaglobulinemia of infancy

Question 20

Leukocyte adherence deficiency is due to a defect in
a.CD 18
b.CD 19
c.CD 20
d.CD 21

Question 21

Which of these diseases would have a dramatic decrease in the CD3+ population?
a.adenosine deaminase deficiency
b.common variable immunodeficiency
c.Bruton's agammaglobulinemia
d.transient hypogammaglobulinemia of infancy

Question 22

Bone marrow transplantation is the treatment of choice for
a.common variable immunodeficiency
b.Bruton's agammaglobulinemia
c.transient hypogammaglobulinemia of infancy
d.X-SCID

Question 23

The nitroblue-tetrazolium test measures
a.B-cell function
b.T-cell function
c.complement function
d.phagocytic cell function

Question 24

Acquired immunodeficiencies can be the result of
a.HIV
b.alcoholism
c.drug use
d.all of the above
e.A and C

Question 25

Selective IgA deficiencies occur mostly in
a.women
b.men
c.people from the Arabian Peninsula
d.people from Asians