Immune Deficiencies

Question 1

What bug? gram (-), oxidase (+), non-fermenter?

Answer 1

Pseudomonas aurigenosa

Question 2

If a white blood cell is unable to reduce NBT (nitro blue tetrazolium), what enzyme is deficient?

Answer 2

NADPH oxidase.

SUPER HIGH YIELD QUESTION ! ! ! !

Question 3

NBT (-) test result means?

Answer 3

they do NOT have NADPH oxidase and susceptible to granulomatous infections.

Question 4

NBT (+) test result means?

Answer 4

they HAVE NADPH oxidase and are not as susceptible to granulomatous infections.

Question 5

What causes Chronic Granulomatous disease?

Answer 5

NADPH oxidase deficiency.

Question 6

What organisms are catalase + that cause granulomatous disease?

Answer 6

“SACKS P”

Serratia
Aspergillus
Candida
Klebsiella
Staph

Pseudomonas

Question 7

Function of Myeloperoxidase?

Answer 7

Turns the H2O2 made by bacteria into Bleach!

its the ‘back up’ plan if NADPH oxidase is not working

Question 8

What makes you more susceptible to a Nisseria Meningitis infection?

Answer 8

MAC deficiency 
(membrane attack complex of the compliment system)

Question 9

DiGeorge Syndrome is due to what defect?

Answer 9

lack of thymus

Question 10

What cells are lacking in DiGeorge?

Answer 10

T-cells

Question 11

Without T-cells, you can’t do what function?

Answer 11

convert IgM to IgG

Question 12

Which Ig will be in huge excess in DiGeorge pts?

Answer 12

IgM (because without T-cells they can’t class switch)

Question 13

What kind of vaccines can a DiGeorge pt NOT get?

Answer 13

live vaccines.
“MMY RSVP-HiB”
Mumps, Measles, Yellow fever
Rubella, Rotovirus, Small pox, Verazella, Polio, HiB

Question 14

Buzzword for DiGeorge?

Answer 14

lack of thymic shadow (SCID also has this)

Question 15

Function of TAP?

Answer 15

proper folding for MHC-1

Question 16

Lack of MHC-1 means you can’t make?

Answer 16

you can’t make CD8

Question 17

Lack of MHC-2 means you can’t make?

Answer 17

you can’t make CD4

Question 18

GVHD (graft vs. host) disease will not occur if you don’t have?

Answer 18

MHC-2

Question 19

Bruton Tyrosine Kinase causes?

Answer 19

no circulation B-cells
-you are lacking tyrosine kinase

(tyrosine kinase is needed to mature the B-cells)

Question 20

What surface marker would be absent in Bruton Tyrosine Kinase?

Answer 20

CD19 or CD20 (B-cell surface marker)

Question 21

Symptoms of Selective IgA deficiency?

Answer 21

repeated sino-pulmonary and GI infections.

CX: you are IgA deficient

Question 22

Leukocyte Adhesion Deficiency caused by?

Answer 22

absence of CD18 “integrin”
“no puss unless she is over 18”

(Integrins bind the WBC tightly to allow the WBC to pass through the endothelial cells in the vessel wall).

Question 23

Symptom of leukocyte adhesion deficiency?

Answer 23

Failure to form pus

Question 24

Chediak - Higashi main symptom?

Answer 24

partial albinism

Question 25

What causes Chediak-Higashi?

Answer 25

phagocyte defect, the granules can’t fuse

Question 26

G6PD deficiency is caused by?

Answer 26

hexose monophosphate shunt enzyme defect

Question 27

G6PD main symptom?

Answer 27

edema

Question 28

Job syndrome is caused by?

Answer 28

TH1 cells can’t make IFN-Y

-PSM don’t respond to chemotactic stimuli

Question 29

Job Syndrome symptoms?

Answer 29

coarse facies, cold abscess, retained primary teeth, eczema