Immune deficiencies

Question 1

congentital granulocytopenia (agranulocytosis)

Answer 1

primary immunodeficiency, caused by block in granulocyte maturation, neutropenia, lacks phagocytosis

Question 2

complement deficiency

Answer 2

primary. caused by mutations/deletions in complement component. Classical pathway and C3 deficiency

Question 3

complement deficiency

Answer 3

primary. caused by mutations/deletions in complement component. Classical pathway anc C3 deficiency

Question 4

SCID

Answer 4

primary. caused by block in lymphocyte develpment. deficiency in T cells and B cells

Question 5

hereditary angeoedema

Answer 5

defect in C1 inhibitor

Question 6

C1 inhibitor

Answer 6

regulates blood clotting system

Question 7

terminal complement components

Answer 7

C5, C6, C7, C8

Question 8

C3 deficiency

Answer 8

failure to clear immune complexes

Question 9

reticular dysgenesis

Answer 9

myeloid and lymphoid stem cells fail to differentiate during hematopoiesis (no b cells, no t cells, no neutrophils)

Question 10

Bare lymphocyte syndrome type1

Answer 10

no HLA class 1 expression - low t cells but normal mitogen

Question 11

Bare lymphocyte syndrome type 2

Answer 11

no HLA class 1 or class 2 expression - low t cells but normal mitogen

Question 12

SCID (severe combined immunodeficiency)

Answer 12

deficient in the production of T-dependent antibodies and CMI - depressed B and T cell counts

Question 13

complement deficiencies

Answer 13

functions of antibody and phagocytes are affected. ex: ()

Question 14

AIDS

Answer 14

HIV-positive serology, CD4+ count less than 200

Question 15

Cell-mediated immunodeficiencies

Answer 15

more sever than b cell deficiencies - impact humoral and cell mediated immunity b/c deficient assistance

Question 16

Cell-mediated immunodeficiencies

Answer 16

more sever than b cell deficiencies - impact humoral and cell mediated immunity b/c deficient assistance

Question 17

DiGeorge syndrome

Answer 17

congenital thymic aplasia, low T cell CD3+ counts

Question 18

Humoral immune deficiencies

Answer 18

deficiencies in b cells

Question 19

bruton’s x-linked agammaglobulinemia

Answer 19

normal pre-b cells but no/low maturation of b-cells and plasma cells

Question 20

x-liinked hyper-IgM syndrome

Answer 20

low/no IgG, IgE and IgA but high IgM - defect in isotype switching

Question 21

IgA deficiency

Answer 21

increased penetration of allergens and subsepuent Ig stimulation

Question 22

Common Variable hypogammaglobulinemia

Answer 22

mature b cells are normal - defect in maturation of functional plasma cells - defects in both cytokine receptor expression and TH2 cytokine production (IL-5)

Question 23

killing defects

Answer 23

chronic granulomatous disease

Question 24

adherence defects

Answer 24

leukocyte adherence deficiency

Question 25

adherence defects

Answer 25

leukocyte adherence deficiency

Question 26

chronic granulomatous disease

Answer 26

defect in glucose-6-phosphate dehydronase and myeloperoxidase (decreased H202 during phagocytosis)

Question 27

lazy leukocyte syndrome

Answer 27

deficiency in chemotactic factors C3a or C5a

Question 28

Leukocyte adherence defieciency

Answer 28

the beta chain of CD18 is defective (inhibited extravasation of cells into extravascular tissue spaces)

Question 29

qualitative deficiencies in neutrophils

Answer 29

defects at any point of the process of phagocytosis may limit the effectiveness of the phagocytic defense system

Question 30

recombinant colony-stimulating factors

Answer 30

G-CSF, GM-CSF = neutropenia treatment

Question 31

congenital agranulocytosis (kostmann syndrome)

Answer 31

complete absence of mature peripheral blood neutrophils (less than 200ul)

Question 32

normal range of neutrophils

Answer 32

3,000 to 6,000/ul

Question 33

opportunistic pathogen

Answer 33

microbe that doesn’t normally cause disease but does so if afforded the opportunity

Question 34

primary immune deficiencies

Answer 34

congenital defects resulting in impaired immune responses

Question 35

primary immune deficiencies

Answer 35

congenital defects resulting in impaired immune responses

Question 36

Most common primary immunodeficiency

Answer 36

IgA deficiency