Immune Deficiencies

Question 1

Primary immunodeficiency is usually of ……origin

Answer 1

Genetic

Question 2

Secondary immunodeficiency is the result of complications from……

1)

2)

3)

Answer 2

1) aging
2) infections
3) SE of immunosupressive therapies (i.e., chemotherapy)

Question 3

Phagocyte (myeloid) defect leads to increase….

Answer 3

bacterial infections

Question 4

Chronic Granulomatous disease

Answer 4

cannot produce superoxide radical

Question 5

Myeloperoxidase deficency

Answer 5

cannot kill intreacellular bacteria

Question 6

Leukocyte Adhesion Deficiency I and II

Answer 6

defects in integrin (I) or sleectin (II) decrease activation and migration of cells to sites of infection

Question 7

Lymphocyte defects lead to ……

Answer 7

broad based immuen deficiencies

Question 8

Why do lymphocyte defects lead to broad based immuen deficiencies?

Answer 8

pathogens can mutate outcompete preformed PAMP and cannot be controleld

Question 9

Define severe combined immunodeficiency

Answer 9

1) rare defect in lymphoid stem cell
2) defects in T cell development
3) Lack of T cell development results in lack of B cell development

Question 10

CD40L is located on….

Answer 10

the CD4 T cell that is bound to CD40 receptor on immature b cell

Question 11

Define Digeorge Syndrome

Answer 11

• thymic aplasia because of defect in 3rd and 4th pharyngeal pouches
• thymic stroma can’t efficently support T cell development and maturation

Question 12

Robust antibody production boosting of ab responses and generation of B cell memory depends on

Answer 12

CD4 Th2 T cell

Question 13

Defects in antibody oposinization causes…

Answer 13

Recurrent bacterial infections

Question 14

Defects in ADCC causes…

Answer 14

Parasitic infections

Question 15

IgA defect leads to….

Answer 15

respiratory, GI, GU infections

Question 16

defects in T cells propsenstiy to produce different types leads to….

Answer 16

1) decreased propensity to produce ab

2) amount and type of ab made

Question 17

Benefits of materanl ab….

Answer 17

partially protects fetus and neonate during the first few months of life

*can obscure defects present in the neonate’s immune system

Question 18

Which Ig is passively transferred from mother to fetus?

Answer 18

IgG

Question 19

*Define X-linked Agammaglobulinemia

Answer 19

• males, because only have one X chromosome
  1) increased infections at 8-9 months
  2) defect in signaling molecule (bruton’s tyrosine kinase)
  3) no mature B cells relased from bone marrow
  4) no GC in lymph nodes, spleen or tonsils, no plasma cells, no serum Ig

Question 20

Defect in bruton’s tyrosine kinase is assoc with

Answer 20

X-linked Agammaglobulinemia

Question 21

X-linked Agammaglobulinemia lacks 3 things

Answer 21

1) no mature B cells relased from bone marrow
2) no gc in lymph nodes, spleen, or tonsil
3) no plasma cells, NO SERUM IG

Question 22

Hyper IgM syndrome is what linked?

Answer 22

70% x-linked

• can have some phenotype from other mutation

Question 23

Hyper IgM Syndrome is associated with….infections

Answer 23

Recurrent bacterial infections

Question 24

List 5 things about hyper IgM

Answer 24

1) 70% X linked: can have same phenotype from other mutations
2) recurrent bacterial infections
3) T cell defect prevents B cell maturation
4) only IgM in the serum
5) no gc in lymph nodes spleen or tonsil

Question 25

Protein calorie malnutrition

Answer 25

1) braod spectrum immunodeficiency

2) secondary immunodefiency

Question 26

Irradation and Chemotherapy

Answer 26

1) decrease cell-mediated immunity

2) leukopenia, thrombocytopenia, neutropenia

Question 27

Cancer Metastases to bone

Answer 27

1) broad spectrum immunodefiency

Question 28

Immunosupressive Drug

Answer 28

1) supress all of immune system

Question 29

Functions of the spleen

Answer 29

1) removal of unwanted elements of the blood

2) major secondary organ of immune system (dc in the spleen trap antigens and present them to Tcells)

Question 30

Source of hematopoietic cells in cases of severe anemia

Answer 30

Spleen

Question 31

Major clinical manifestation of removal of spleen

Answer 31

1) increased susceptibility to disseminated infection w/ encapsulated bacteria
- penumococcus
- meningococcus
- Haemophilus Influenzae