Immune Deficiencies Flashcards
What bug? gram (-), oxidase (+), non-fermenter?
Pseudomonas aurigenosa
If a white blood cell is unable to reduce NBT (nitro blue tetrazolium), what enzyme is deficient?
NADPH oxidase.
SUPER HIGH YIELD QUESTION ! ! ! !
NBT (-) test result means?
they do NOT have NADPH oxidase and susceptible to granulomatous infections.
NBT (+) test result means?
they HAVE NADPH oxidase and are not as susceptible to granulomatous infections.
What causes Chronic Granulomatous disease?
NADPH oxidase deficiency.
What organisms are catalase + that cause granulomatous disease?
“SACKS P”
Serratia Aspergillus Candida Klebsiella Staph
Pseudomonas
Function of Myeloperoxidase?
Turns the H2O2 made by bacteria into Bleach!
its the ‘back up’ plan if NADPH oxidase is not working
What makes you more susceptible to a Nisseria Meningitis infection?
MAC deficiency (membrane attack complex of the compliment system)
DiGeorge Syndrome is due to what defect?
lack of thymus
What cells are lacking in DiGeorge?
T-cells
Without T-cells, you can’t do what function?
convert IgM to IgG
Which Ig will be in huge excess in DiGeorge pts?
IgM (because without T-cells they can’t class switch)
What kind of vaccines can a DiGeorge pt NOT get?
live vaccines.
“MMY RSVP-HiB”
Mumps, Measles, Yellow fever
Rubella, Rotovirus, Small pox, Verazella, Polio, HiB
Buzzword for DiGeorge?
lack of thymic shadow (SCID also has this)
Function of TAP?
proper folding for MHC-1
Lack of MHC-1 means you can’t make?
you can’t make CD8
Lack of MHC-2 means you can’t make?
you can’t make CD4
GVHD (graft vs. host) disease will not occur if you don’t have?
MHC-2
Bruton Tyrosine Kinase causes?
no circulation B-cells
-you are lacking tyrosine kinase
(tyrosine kinase is needed to mature the B-cells)
What surface marker would be absent in Bruton Tyrosine Kinase?
CD19 or CD20 (B-cell surface marker)
Symptoms of Selective IgA deficiency?
repeated sino-pulmonary and GI infections.
CX: you are IgA deficient
Leukocyte Adhesion Deficiency caused by?
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).
Symptom of leukocyte adhesion deficiency?
Failure to form pus
Chediak - Higashi main symptom?
partial albinism
What causes Chediak-Higashi?
phagocyte defect, the granules can’t fuse
G6PD deficiency is caused by?
hexose monophosphate shunt enzyme defect
G6PD main symptom?
edema
Job syndrome is caused by?
TH1 cells can’t make IFN-Y
-PSM don’t respond to chemotactic stimuli
Job Syndrome symptoms?
coarse facies, cold abscess, retained primary teeth, eczema
