Immunodeficiency

Question 1

humoral immunodeficiency

Answer 1

antibodies and b-cells improper or not formed.

clinical : pneumonia, ear infections.

Question 2

cellular immunodeficiency

Answer 2

fungal, protozoa, viral.

healthy patient not affected

Question 3

combined

Answer 3

both b & t cells affected = severe infection

Question 4

non-specific

Answer 4

innate immunity= phagocytes, neutrophils.

recurring pyogenic bacteria, skin infection, inappropriate/inefficient wound healing.

Question 5

x-linked aggamglobulinemia (bruton’s syndrome)

Answer 5

in maturation of b cell. genetic defect. no b-cells, no Ab

clinical: penumonia, bacterial meningitis septisemia, ear infection
treat: IVIG

Question 6

IgA Deficiency & Hyper-IgM Syndrome

Answer 6

problem in switching types of Ig produced.

IgA - immune = no IgA. clinical = increase mucosal infections, bacterial infections

hyper-IgM = defect in t-b cell interaction = no Ig isotype switching. 
immune = only IgM. 
clinical = bacterial infection - can't fight pathogen.

Question 7

common variable immunodeficiency

Answer 7

plasma cell - affected ability to produce Ab and convert to memory B.
immune = low ab level
clinic = pyogenic bacteria

Question 8

DiGeorge Syndrome

Answer 8

congenital thymic aplasia - no thymus.
immune = no t-cells, no where to develop
clinical = cant regulate Ca2+ in blood, congenital cardiac disease.

Question 9

bare lymphocyte syndrome

Answer 9

last maturation step.
type 1 = affect MHCI - (on CD8) decreased
clinical = increased viral infections
type 2 = affect MHC2 - (on all cells) decresed = fewer helper t-cells = innate immunity doesnt work

Question 10

SCID

Answer 10

severe combined immunodeficiency
immune: decrease # of b- and t-cells.
clinical = susceptible to everything = bubble boy

Question 11

non-specific

Answer 11

LAD 1&2 = lost ability to adhere to endothelium - cant get into tissue

chediak-higashi = defect in fusion of vesicles w lysosome
chronic granulomatuus disease = cant complete phagocytosis.

clinical = increase pyogenic bacteria, decrease ability to heal wound

Question 12

secondary immunodeficiency

Answer 12

immune system attacked. immunoglobulin loss. neoplasia, aging, iatrogenic transmission

Question 13

AIDS

Answer 13

virus attacks CD4 cells destroy cellular immunity.
lymphopenia = decrease CD4, altered b-cell activation, altered macrophage
clinical = opportunistic bacteria take advantage.