Immunodeficiencies

Question 1

Immunodeficiency diseases are defective host defense systems classified as… or …

Answer 1

congenital (primary) or acquired (secondary)

Question 2

Primary immunodeficiencies are classified in what four ways?

Answer 2

• B cell or humoral
• T cell or cellular
• Defects of phagocytes
• Abnormalities of the complement system

Question 3

People with antibody deficiency diseases are inordinately susceptible to…

Answer 3

recurrent bacterial infections

Question 4

Bruton X-linked Agammaglobulinemia typically appears in which sex and age?

Answer 4

Boys <1 year old (manifests early)

Question 5

Bruton X-linked Agammaglobulinemia is characterized by a lack of… in …

Answer 5

lack of immature B cells in periphery and plasma

Question 6

Which primary immunodeficiency is a genetic defect on long arm of X chromosome rendering B cells incapable of maturation?

Answer 6

Bruton X-linked Agammaglobulinemia

Question 7

With Bruton X-linked Agammaglobulinemia, patients develop recurrent…

Answer 7

infections of mucosal surfaces

Question 8

What type of diagnosis is required for Bruton X-linked Agammaglobulinemia?

Answer 8

Lab diagnosis

Question 9

DiGeorge syndrome is caused by defective development of the third and fourth pharyngeal pouches, which give rise to… and influence…

Answer 9

thymic epithelium and parathyroid glands and influence conotruncal cardiac development

Question 10

In the absence of functional thymus… is interrupted

Answer 10

T-cell maturation is interrupted at the pre-T cell stage

Question 11

Which cells are effected by DiGeorge Syndrome?

Answer 11

T cells

Question 12

Those with DiGeorge Syndrome have frequent…

Answer 12

recurrent infections

Question 13

What is Severe Combined Immunodeficiency?

Answer 13

The worst of primary immunodeficiencies; encompasses over 20 disorders associated by deficiencies in T-cell and B-cell development and function

Question 14

Severe Combined Immunodeficiency includes…

Answer 14

X-linked, autosomal recessive

Question 15

Patients affected by Severe Combined Immunodeficiency present in the first few months of life with…

Answer 15

recurrent, often severe infections, diarrhea, and failure to thrive

Question 16

What is the treatment for Severe Combined Immunodeficiency?

Answer 16

Stem cell graft treatment

Question 17

Which primary immunodeficiency leads to spider veins?

Answer 17

Immunodeficiency with ataxia telangiectasia

Question 18

Which primary immunodeficiency has increased susceptibility to infection, specifically frequent sinopulmonary infections?

Answer 18

Immunodeficiency with ataxia telangiectasia

Question 19

How does immunodeficiency with ataxia telangiectasia affect DNA, T cells, and immunoglobulins?

Answer 19

Damages DNA by affecting repair, lowers T cell numbers as well as IgG, IgA, and IgE

Question 20

Which primary immunodeficiency is an autosomal recessive disorder demonstrating increased susceptibility to infection by pyogenic bacteria?

Answer 20

Chediak-Higashi Syndrome

Question 21

Due to defect in membrane organelles, those with Chediak-Higashi Syndrome have defective…

Answer 21

fusion of lysosomes and phagosomes

Question 22

Patients with Chediak-Higashi Syndrome will have reduced ability to… leading to…

Answer 22

reduced ability to kill ingested microbes, leading to recurrent infections with pus

Question 23

Which primary immunodeficiency occurs with frequent albinism of eyes and skin?

Answer 23

Chediak-Higashi Syndrome

Question 24

What is Wiskott-Aldrich Syndrome?

Answer 24

X-linked disorder demonstrating increased susceptibility to infection, especially S. aureus

Question 25

Which cells are affected by Wiskott-Aldrich Syndrome?

Answer 25

T and B cell numbers and functions reduced as well as immunoglobulins Platelets abnormal and reduced

Question 26

What would be the effect of abnormal and reduced platelets that come with Wiskott-Aldrich Syndrome?

Answer 26

Prolonged bleeding, easy bruising

Question 27

LAD type I has deficiencies in... Patients have...

Answer 27

deficiencies in integrins Patients have localized bacterial infections which may be life-threatening

Question 28

LAD type II has deficiencies in... Patients have...

Answer 28

deficiencies in selectins Patients life-threatening infections and severe developmental delay

Question 29

LAD I and II are deficiencies in...

Answer 29

leukocyte adhesion

Question 30

With LAD I and II, flow cytometry shows...

Answer 30

elevated WBC count with confirmation of molecular deficiency

Question 31

What is physiologic sequelae?

Answer 31

Inability to build immune system General malnutrition, vitamin and mineral deficiency

Question 32

Ionizing radiation, chemotherapeutics, anti-inflammatory drugs, and immunosuppressants are all examples of...

Answer 32

therapeutic treatments for secondary immunodeficiency

Question 33

Ionizing radiation damages bone marrow which will affect...

Answer 33

B-cells

Question 34

What type of drug is prednisone?

Answer 34

Anti-inflammatory

Question 35

What are three infectious examples of secondary immunodeficiency?

Answer 35

* HIV * EBV * Schistosoma

Question 36

With HIV, what aspect of the immune system is low?

Answer 36

CD4+ which is important for T-cells

Question 37

What happens when a patient is infected with EBV?

Answer 37

WBCs are transformed to cancer cells

Question 38

What is schistosoma?

Answer 38

Parasites consume immune system which can cause cancer

Question 39

Cancers like multiple myeloma, Waldenstrom's macroglobulinemia, and leukemias and lymphomas are all examples of what type of immunodeficiency?

Answer 39

Secondary

Question 40

What is the effect of multiple myeloma?

Answer 40

Destroyed bone marrow which produces immune cells

Question 41

Multiple myeloma's "lesser cousin" is...

Answer 41

Waldenstrom's macroglobulinemia

Question 42

Leukemias and lymphomas are all...

Answer 42

immune cell cancers where cancer takes over the immune system