Immunodeficiency

Question 1

CGD

Answer 1

Decreased phagocyte fxn - defect in NADPH oxidase
X linked
Susceptible to catalase positive (S. aureus, Serratia, Aspergillus)
Recurrent skin abscesses, severe prolonged pneumonia, bone
NBT test

Question 2

SCID

Answer 2

Absence of functional T-cells and consequently B cells
Severe recurrent infections
Usually die in first year unless have HSC
transplant
Low or absent T cells and NK cells and non-functional B cells.

Question 3

IgA Def

Answer 3

Limited increase in infection because of IgM compensation
Low IgA
Common

Question 4

Complement Def

Answer 4

C1-4 deficiency present with pyogenic infections
C5-9 deficiency present with serious Neisseria infections
C2 most common
Decreased CH50

Question 5

CVID

Answer 5

Most common serious primary defect in adults inadequate antibody formation
Onset in teens or 20s
Increased risk of non-Hodgkins lymphoma and gastric cancer
Recurrent pyogenic sinopulmonary infections (S. pneumo)
Chronic diarrhea (Giardia, C. jejuni) GI lymphoid hyperplasia, increased bacteremia
Some PCP, fungi, mycobacteria, recurrent HSV
Decreased IgG, IgM, IgA
Normal number of B cells and T
cells

Question 6

Hyper IgE

Answer 6

aka Jobs
Often from STAT3 gene mutation
FATED: coarse or leonine facies, cold staph abscesses, retained primary teeth, increased IgE, and dermatologic

Question 7

HIV Onset

Answer 7

Acute febrile illness, mono-like illness +/- aseptic meningitis  Maculopapular rash, mucosal ulcerations
Usually 2-3 wks after HIV exposure in >50% - risk transmission is high
Enter using receptor & co-receptor (CCR5 & CXCR4)

Question 8

Granulocytopenia Opportunistic Infxns

Answer 8

Chemo/Radiation

Gram negs & Staph

Question 9

HIV classification CD4 levels

Answer 9

CD4 > 500
Intermediate 200-500
Advanced/AIDS:

Question 10

Pneumocystis Pneumonia

Answer 10

Yeast like fungus called P. jirovecci
 PCP causes elevated LDL
Usually bilateral, interstitial
Positive fluorescent antibody
GMS stain w/lavage
Tx: Bactrim (3 wks), If not then Clinadamycin + Primiquine or IV pentamidine

Question 11

Cellular Immune Dysfxn

Answer 11

AIDS, age, smoking

Mycobacterium tuberculosis & avium, listeria

Question 12

Humoral Dysfxn Opportunistic Infxns

Answer 12

Agammaglobulinemia, splenectomy

Encapsulated (S. pneumo, meningococci)

Question 13

Foreign Body Opportunistic Infxns

Answer 13

Gram negs & staph

Question 14

Surgery Opportunistic infxns

Answer 14

Staph, e. coli, pseudomonas

Question 15

Iron and bacteria

Answer 15

Most need iron for growth

So iron binding proteins keep them from using the iron = defense

Question 16

Acinetobactor

Answer 16

Soil and water
Resistant
Outbreaks often in ICUs and ill patients
Caused severe infection in wounded soldiers during the Iraq war

Question 17

Pseudomona aeruginosa

Answer 17

Premier opportunist infxn
Likes wet environments
skin, ear (diabetes), sepsis, GI (premies), pulmonary