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Flashcards in L 22 Deck (45)
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1

What are primary immuno-def?

Inherited!
Many won't survive to term

2

If you damage this immune cell, you will always get systemic problems. Which one?

Th = brain!

3

B/T/Innate: losing this will yield recurrent bacterial infections.

B cell def
Sometimes innate def

4

B/T/Innate: losing this will yield recurrent viral infections.

T cell def

5

Why might you seen auto-immunity WITH immuno-def?

Th sees there is a defect somewhere in the system
Tries to compensate by ↑cytokines to activate those missing cells
Instead, causes abnormally ↑activity other cells

6

When is oral thrush normal vs patho?

Normal = childhood or immune suppressed adults
Abnormal = adults --> immuno-def

7

A young kid presents after having a few infections recently. They were treated with antibiotics and resolved, but mom is concerned. Labs show slightly ↓IgG. What is your diagnosis and treatment?

Transient hypoglobulinemia of infancy
DO NOTHING - resolves on own
↓in mom's IgG - this is a lull time as kid makes own IgG

8

A 30 yo patient presents after having multiple different infections. Labs show ↓Ig & no plasma cells. What is your diagnosis? Treatment?

Common variably immuno-def
1. Ig replacement
2. Bone marrow transplant

9

What is the pathophys of CVID?

Unknown mechansim
Normal/low B cells
CANT become plasma cells! ↓↓↓Ig (but there is some!)
Therefore: recurrent infections of all types

10

What are patients with ↓B cell fxn at ↑risk for?

Autoimmune & cancer

11

If transient hypoglobulinemia doesn't resolve on its own, what should you think about?

Burton's agammaglobinemia

12

A 9 month boy has had recurrent otitis media & pneumococcal pneumoia in the past 3 mos. What disease are you thinking about?

Burtons A-G-G

13

What is the pathophys of Burton's A-G-G?

Mutated Burton's tyrosine kinase
PreB cells can't progress

14

What are the values for Burton's A-G-G?
Pro-B cells
B cells
Ig
T cells

Normal pro-B & T
Low B & Ig

15

What are the symptoms of IgA def?

Pulm & GI infections
(Bronchitis & pneumonia duo)

16

How do you treat IgA def?

Give antibiotics for infections as they present

17

What should you 100% avoid for IgA def patients?

BLOOD TRANSFUSION

18

What immune cell fights Epstein-Barr?

B cells
Via CD21
Beer at the Bar when you're 21

19

What should you be thinking about for a patient presenting with ↑ B cells with ↓Ig and mono?

X-linked lympho-proliferative syndrome
EBV infecting B cells
Making them proliferate --> tumor

20

A child presents with craniofacial & cardiovascular defects and tetany. What are you thinking of? Treatment?

DiGeorge's Syndrome
Hypo-thyroid = NO T CELLS
--> hypo-calciemia
Treat: fetal thymic transplant

21

What causes DiGeorge's syndrome? Why does this happen?

3rd and 4th pharyngeal pouches disturbed during development
Due to:
Delete chromosome 22
OR
Teratogens

22

What is a skin infection that is chronic if you are lacking T cells or IL 17/R? What age is this infection common?

Chronic muco-cutaneous candidiasis
Childhood

23

What is type 1 hyper IgM? Inheritance? Histo?

X linked --> BOYS
X CD40/CD40 L = X B/T interactions
T cells can't help B cells differentiate
No isotype switching
NO GERMINAL CENTERS

24

What is type 2 hyper IgM? Inheritance? Histo?

A.recessive
X AID --> X SHM & CSR
LARGE germinal centers

25

What are the 5 ways you can get SCID?

1. X linked - gamma chain
2. X JAK 3 --> X IL 7 --> X T cells --> nonfxn B
3. X ADA - accumulated adenosine
4. X PNP
5. reticular dysgenesis
6. Omen syndrome

26

What is the difference between PNP & ADA SCID?

ADA - ↓T & B
PNP - ↓T, normal B

27

What is unique about ADA SCID?

Defected cartilage formation

28

What is unique about X linked SCID?

Normal B cells - ↓IgG b/c no SHM/CSR

29

What is Omen syndrome?

X RAG1/2
X VDJ recomb
↑IgE - allergic symptoms with immuno-def

30

What enzyme is missing for radioactive Omen?

Artemis
No NHEJ --> can't DNA repair

31

Which receptor is most important for X linked SCID?

IL 2

32

A patient presents with Staph aureus infection with both skin infection and sub-q abscesses. Labs show ↑IgE. What are you thinking of? What is the mutation? What other symptoms should you see?

Job Syndrome
STAT3 mutation
Weird teeth

33

What is the difference between Omen and Job patients?

Omen won't survive to the ages Job will

34

A young boy presents with eczema and purpura. He has a history of repeated Step pneumo & staph aureus infections. What are you thinking?

Wiskott-Aldrich syndrome

35

What is Wiskott-Aldrich syndrome? Inheritance?

X linked
Altered WBC & platelet cytoskeleton
↓T & B cells

36

You have an immuno-def ataxic patient. What disease do they have?

Ataxia telangiectasia

37

What is A-T?

No DNA repair
T & B defects
1. Cerebellar atrophy
2. Occular telangiectasia = dilated blood vessels in eye
3. Radiosensitive

38

What kind of deficiency presents with skin infections & osteomyelitis?

Phagocyte defects

39

Albino patients with bleeding & peripheral neuropathy.

Chediak-Higashi Syndrome
Dysfxn microtubles - phagocytes can't phagocytose!!

40

Young boy with granulomas in the skin, GI, GU and history of fungal & bacterial infections.

CGD
X linked
X NAPDH oxidase

41

What is the difference between type 1 & 2 leukocyte adhesion deficiency?

1. Die young
X CD 18 (LFA = integrin)
2. Survive w/ mental retardation & neuro problems
X Selectins

42

Which hyper IgM is boys only?

Type 1 - CD40L

43

Which hyper IgE is in older patients?

Job
NOT Omen

44

Which disease is radiosensitive in adult patients?

Ataxia telagniectasia
NOT Omen

45

What is the Wiskott-Aldrich syndrome triad?

Thrombocytopenia
Eczema
Recurrent infections