UAMS Immunology Exam #4 UPDATED
ACTUAL Questions and CORRECT
Answers
Transient hypogammaglobulinemia of infancy - CORRECT ANSWER - What condition
is classified as a B cell deficiency that has prolonged low Ig levels, but usually normalize
between 9-15 months? Normal B cells are present but may be due to delayed maturation of Th
cells.
X-linked Bruton's agammaglobulinemia. Caused by Bruton's tyorsine kinase and results in
failure of Vh gene rearrangement. Recurrent otitis and areas of cellulitis in the diaper area and
skin lesions. - CORRECT ANSWER - What disease is classified as a B cell deficiency
that is linked to chromosome X and is seen almost exclusively in males due to development
being blocked at the pro-B-cell stage to the pre-B-cell stage and lack all classes of Ig? What
causes this? What does it result in? What is the clinical manifestation?
If there is persistence of abnormal histology of lymphoid tissue beyond 2 years of age, it is x-
linked bruton's most likely. - CORRECT ANSWER - How can x-linked bruton's be
differentiated from transient hypogammaglobuinemia?
IgA deficiency. Some produce anti-IgA ABYs following blood transfusion. - CORRECT
ANSWER - What is the most common primary immunodeficiency that is classified as a B
cell deficiency? Most patients are asymptomatic. What do some patients produce that causes
anaphylaxis?
Common variable immunodeficiency. Clinical manifestation presents as malabsorption and
diarrhea, malignancies, and autoimmune disorders. Diagnosed by low serum IgG in patient with
recurrent bacterial infections. Treated with injection of IV Ig preparations and antibiotics as
needed. - CORRECT ANSWER - Which condition is classified as a B cell deficiency that
is characterized by a normal # of B cells that cannot differentiate into plasma cells? What is the
clinical manifestation? How is it diagnosed? What is the treatment?
Cellular immunity deficiencies - CORRECT ANSWER - What type of deficiency requires
a bone marrow transplant and as a result has problems with graft vs. host disease?
,Digeorge anomaly. Embryonic devleopment defect that affects the thymus primarily due to an
insufficient number of T cells. Treated with a fetal thymus and BM transplant and thymic
hormones. - CORRECT ANSWER - What is classified as a cellular immunity deficiency
and is characterized by a missing portion of chromosome 22? What is the defect and treatment?
Purine nucleoside phosphorylase deficiency or PNP. The number of T cells decrease as a toxic
purine metabolite accumulates. - CORRECT ANSWER - What condition is due to a
defect in the enzyme involved in purine metabolism and classified as a T cell deficiency? What
is the mechanism?
Severe combined immunodeficiency or SCID. X-linked SCID is most common and affects
normal signaling between cells and halts cell maturation. Autosomal recessive SCID. Adenosine
deaminase or ADA deficiency. - CORRECT ANSWER - What condition is the most
severe congenital immune deficiency? What is the most common defect? What defect is when
the child inherits two defective copies? What is the deficiency?
Wiskott-aldrich syndrome or WAS. Clinical manifestation presents as triad of
immunodeficiency, eczema, and thrombocytopenia. - CORRECT ANSWER - Which
condition is caused by an X-linked protein defect and is usually lethal in childhood due to
infection, malignancy, or hemorrhage? What is the clinical manifestation?
Ataxia-telangiectasia or AT. Presents with muscle incoordination and capillary dilation. -
CORRECT ANSWER - Which condition is caused by a defect in the gene recombination
process? What is the clinical manifestation?
Chronic granulomatous disease or CGD. Defects affect production of reactive oxygen forms and
treated with granulocyte transfusions, cytokine administration, and BM transplantation. -
CORRECT ANSWER - Which condition usually causes death in early childhood due to
defective microbial killing that results in recurrent infections? What is the defect and treatment?
Myeloperoxidase deficiency or MPO. - CORRECT ANSWER - Which condition is the
most common neutrophil abnormality that generally does not cause disease but can cause the
occasional disseminated candidiasis in diabetics?
, Leukocyte adhesion molecule deficiency. Presents as delayed wound healing and chronic skin
infections. - CORRECT ANSWER - Which condition is characterized by a defect in
adhesion receptors in PMNs, monocytes, and T cells and results in abnormal adhesion, motility,
chemotaxis, and endocytosis? What is the clinical manifestation?
Hypersensitivity. Occurs when responses to foreign AGNs become deregulated or uncontrolled
or when responses are directed against self AGNs (autoimmunity). - CORRECT
ANSWER - Which immune response harms the host? When does this occur?
Type I immediate hypersensitivity. - CORRECT ANSWER - Which classification type is
ABY mediated where cell bound ABYs react with AGN to release active substances?
Type II ABY mediated diseases - CORRECT ANSWER - Which classification type is
ABY mediated where free ABYs react with AGN on cell surface?
Type III immune complex mediated diseases - CORRECT ANSWER - Which
classification type is ABY mediated where ABY reacts with soluble AGN and results in
complexes being precipitated in tissues?
Type IV T cell mediated delayed diseases - CORRECT ANSWER - Which classification
type is T cell mediated where sensitized T cells are responsible for symptoms?
ABY - I, II, and III. Immediate type hypersensitivity. Symptoms occur within hours to minutes.
T cell - IV. Delayed type hypersensitivity. Symptoms occur 24-48 hours after contact with AGN.
- CORRECT ANSWER - Which classification types are ABY mediated and which ones
are T cell mediated? Are these immediate or delayed? When do symptoms occur?
Type I - CORRECT ANSWER - Which classification type is anaphylatic?
Atopic AGNs or allergens - CORRECT ANSWER - What triggers the formation of IgE?
ACTUAL Questions and CORRECT
Answers
Transient hypogammaglobulinemia of infancy - CORRECT ANSWER - What condition
is classified as a B cell deficiency that has prolonged low Ig levels, but usually normalize
between 9-15 months? Normal B cells are present but may be due to delayed maturation of Th
cells.
X-linked Bruton's agammaglobulinemia. Caused by Bruton's tyorsine kinase and results in
failure of Vh gene rearrangement. Recurrent otitis and areas of cellulitis in the diaper area and
skin lesions. - CORRECT ANSWER - What disease is classified as a B cell deficiency
that is linked to chromosome X and is seen almost exclusively in males due to development
being blocked at the pro-B-cell stage to the pre-B-cell stage and lack all classes of Ig? What
causes this? What does it result in? What is the clinical manifestation?
If there is persistence of abnormal histology of lymphoid tissue beyond 2 years of age, it is x-
linked bruton's most likely. - CORRECT ANSWER - How can x-linked bruton's be
differentiated from transient hypogammaglobuinemia?
IgA deficiency. Some produce anti-IgA ABYs following blood transfusion. - CORRECT
ANSWER - What is the most common primary immunodeficiency that is classified as a B
cell deficiency? Most patients are asymptomatic. What do some patients produce that causes
anaphylaxis?
Common variable immunodeficiency. Clinical manifestation presents as malabsorption and
diarrhea, malignancies, and autoimmune disorders. Diagnosed by low serum IgG in patient with
recurrent bacterial infections. Treated with injection of IV Ig preparations and antibiotics as
needed. - CORRECT ANSWER - Which condition is classified as a B cell deficiency that
is characterized by a normal # of B cells that cannot differentiate into plasma cells? What is the
clinical manifestation? How is it diagnosed? What is the treatment?
Cellular immunity deficiencies - CORRECT ANSWER - What type of deficiency requires
a bone marrow transplant and as a result has problems with graft vs. host disease?
,Digeorge anomaly. Embryonic devleopment defect that affects the thymus primarily due to an
insufficient number of T cells. Treated with a fetal thymus and BM transplant and thymic
hormones. - CORRECT ANSWER - What is classified as a cellular immunity deficiency
and is characterized by a missing portion of chromosome 22? What is the defect and treatment?
Purine nucleoside phosphorylase deficiency or PNP. The number of T cells decrease as a toxic
purine metabolite accumulates. - CORRECT ANSWER - What condition is due to a
defect in the enzyme involved in purine metabolism and classified as a T cell deficiency? What
is the mechanism?
Severe combined immunodeficiency or SCID. X-linked SCID is most common and affects
normal signaling between cells and halts cell maturation. Autosomal recessive SCID. Adenosine
deaminase or ADA deficiency. - CORRECT ANSWER - What condition is the most
severe congenital immune deficiency? What is the most common defect? What defect is when
the child inherits two defective copies? What is the deficiency?
Wiskott-aldrich syndrome or WAS. Clinical manifestation presents as triad of
immunodeficiency, eczema, and thrombocytopenia. - CORRECT ANSWER - Which
condition is caused by an X-linked protein defect and is usually lethal in childhood due to
infection, malignancy, or hemorrhage? What is the clinical manifestation?
Ataxia-telangiectasia or AT. Presents with muscle incoordination and capillary dilation. -
CORRECT ANSWER - Which condition is caused by a defect in the gene recombination
process? What is the clinical manifestation?
Chronic granulomatous disease or CGD. Defects affect production of reactive oxygen forms and
treated with granulocyte transfusions, cytokine administration, and BM transplantation. -
CORRECT ANSWER - Which condition usually causes death in early childhood due to
defective microbial killing that results in recurrent infections? What is the defect and treatment?
Myeloperoxidase deficiency or MPO. - CORRECT ANSWER - Which condition is the
most common neutrophil abnormality that generally does not cause disease but can cause the
occasional disseminated candidiasis in diabetics?
, Leukocyte adhesion molecule deficiency. Presents as delayed wound healing and chronic skin
infections. - CORRECT ANSWER - Which condition is characterized by a defect in
adhesion receptors in PMNs, monocytes, and T cells and results in abnormal adhesion, motility,
chemotaxis, and endocytosis? What is the clinical manifestation?
Hypersensitivity. Occurs when responses to foreign AGNs become deregulated or uncontrolled
or when responses are directed against self AGNs (autoimmunity). - CORRECT
ANSWER - Which immune response harms the host? When does this occur?
Type I immediate hypersensitivity. - CORRECT ANSWER - Which classification type is
ABY mediated where cell bound ABYs react with AGN to release active substances?
Type II ABY mediated diseases - CORRECT ANSWER - Which classification type is
ABY mediated where free ABYs react with AGN on cell surface?
Type III immune complex mediated diseases - CORRECT ANSWER - Which
classification type is ABY mediated where ABY reacts with soluble AGN and results in
complexes being precipitated in tissues?
Type IV T cell mediated delayed diseases - CORRECT ANSWER - Which classification
type is T cell mediated where sensitized T cells are responsible for symptoms?
ABY - I, II, and III. Immediate type hypersensitivity. Symptoms occur within hours to minutes.
T cell - IV. Delayed type hypersensitivity. Symptoms occur 24-48 hours after contact with AGN.
- CORRECT ANSWER - Which classification types are ABY mediated and which ones
are T cell mediated? Are these immediate or delayed? When do symptoms occur?
Type I - CORRECT ANSWER - Which classification type is anaphylatic?
Atopic AGNs or allergens - CORRECT ANSWER - What triggers the formation of IgE?
