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Immunology > L 22 > Flashcards

L 22 Flashcards

1
Q

What are primary immuno-def?

A

Inherited!

Many won’t survive to term

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2
Q

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

A

Th = brain!

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3
Q

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

A

B cell def

Sometimes innate def

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4
Q

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

A

T cell def

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5
Q

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

A

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

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6
Q

When is oral thrush normal vs patho?

A 
Normal = childhood or immune suppressed adults
Abnormal = adults --> immuno-def
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7
Q

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?

A

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

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8
Q

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

A

Common variably immuno-def

  1. Ig replacement
  2. Bone marrow transplant
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9
Q

What is the pathophys of CVID?

A

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

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10
Q

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

A

Autoimmune & cancer

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11
Q

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

A

Burton’s agammaglobinemia

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12
Q

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

A

Burtons A-G-G

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13
Q

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

A

Mutated Burton’s tyrosine kinase

PreB cells can’t progress

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14
Q 
What are the values for Burton's A-G-G?
Pro-B cells
B cells
Ig
T cells
A

Normal pro-B & T

Low B & Ig

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15
Q

What are the symptoms of IgA def?

A

Pulm & GI infections

Bronchitis & pneumonia duo

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16
Q

How do you treat IgA def?

A

Give antibiotics for infections as they present

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17
Q

What should you 100% avoid for IgA def patients?

A

BLOOD TRANSFUSION

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18
Q

What immune cell fights Epstein-Barr?

A

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

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19
Q

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

A

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

20
Q

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

A

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

21
Q

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

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

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

A

Chronic muco-cutaneous candidiasis

Childhood

23
Q

What is type 1 hyper IgM? Inheritance? Histo?

A 
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
Q

What is type 2 hyper IgM? Inheritance? Histo?

A

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

25
Q

What are the 5 ways you can get SCID?

A
  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
Q

What is the difference between PNP & ADA SCID?

A

ADA - ↓T & B

PNP - ↓T, normal B

27
Q

What is unique about ADA SCID?

A

Defected cartilage formation

28
Q

What is unique about X linked SCID?

A

Normal B cells - ↓IgG b/c no SHM/CSR

29
Q

What is Omen syndrome?

A

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

30
Q

What enzyme is missing for radioactive Omen?

A

Artemis

No NHEJ –> can’t DNA repair

31
Q

Which receptor is most important for X linked SCID?

A

IL 2

32
Q

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?

A

Job Syndrome
STAT3 mutation
Weird teeth

33
Q

What is the difference between Omen and Job patients?

A

Omen won’t survive to the ages Job will

34
Q

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

A

Wiskott-Aldrich syndrome

35
Q

What is Wiskott-Aldrich syndrome? Inheritance?

A

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

36
Q

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

A

Ataxia telangiectasia

37
Q

What is A-T?

A 
No DNA repair 
T & B defects
1. Cerebellar atrophy 
2. Occular telangiectasia = dilated blood vessels in eye
3. Radiosensitive
38
Q

What kind of deficiency presents with skin infections & osteomyelitis?

A

Phagocyte defects

39
Q

Albino patients with bleeding & peripheral neuropathy.

A

Chediak-Higashi Syndrome

Dysfxn microtubles - phagocytes can’t phagocytose!!

40
Q

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

A

CGD
X linked
X NAPDH oxidase

41
Q

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

A
  1. Die young
    X CD 18 (LFA = integrin)
  2. Survive w/ mental retardation & neuro problems
    X Selectins
42
Q

Which hyper IgM is boys only?

A

Type 1 - CD40L

43
Q

Which hyper IgE is in older patients?

A

Job

NOT Omen

44
Q

Which disease is radiosensitive in adult patients?

A

Ataxia telagniectasia

NOT Omen

45
Q

What is the Wiskott-Aldrich syndrome triad?

A

Thrombocytopenia
Eczema
Recurrent infections

Immunology (25 decks)

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