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Block 6; Week 1-Michelle > Hogan-Clinical Primary Immuno > Flashcards

Hogan-Clinical Primary Immuno Flashcards

1
Q

50% of immunodeficiencies have to do with what?

A

B cells; antibody defects

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2
Q

30% of immunodeficiencies have to do with what?

A

T cells; T cells defects & combined immunodeficiencies

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3
Q

20% of immunodeficiencies have to do with what?

A

innate immunity–18% phagocytic defects, 2% complement deficiencies

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4
Q

Which immune things are present in a neonate?

A 
WBCs (including phagocytes)
parts of complement
NK fcn
T cell fcn
a little IgM
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5
Q

Which immune things take time to develop in a neonate?

A

B cells & their antibodies (6-24 months away)

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6
Q

If you have a T cell deficiency at birth…what other deficiency will you likely have?

A

B cell. T cells help out the T cells.

portions of the complement system dependent on antibodies

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7
Q

If you are worried that a neonate has an infection & you want to test immune markers for that…which immunoglobulin do you look for?

A

IgM b/c the production of this begins shortly before birth. The only one really intact.

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8
Q

What do you do if a baby comes back with IgA levels of 0?

A

Nothing! So what…baby isn’t making IgA yet.

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9
Q

How long are we passively protected by maternal antibody?

A

6 mo

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10
Q

T/F Autoimmune & immunodeficiency problems are completely separate & should not be considered potentially linked.

A

False.
They both have to do with B cells. If a pt is suspected to have immunodeficiency & has a family hx of autoimmune disease-pay attention. Immuno can come before auto or auto before immune.

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11
Q

If you have problems with your innate immunity–what types of infections might you get?

A

Lymphadenitis, osteomyelitis, pneumonia, and sepsis

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12
Q

If you have problems with phagocytes–what types of infections might you get? Why?

A

Phagocytes are non-discriminatory. Wide range of possibilities. Both Gram+ and Gram- and yeast/fungal organisms are attacked

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13
Q

When you get infections from organisms that produce catalase…which disorder might you have?

A

a problem with your neutrophils (a phagocyte)

chronic granulomatous disease

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14
Q

Give some examples of infections you might get with CGD.

A

Staphylococci aureus (Gram + in clusters)
Pseudomonas aeroginosa (Gram -)
Aspergillus fumigatus (fungal)
Candida (yeast)
Enterbacteriaceae (Klebsiella and Serratia) Gram-
Others: Nocardia (Gram + rod) /Listeria (Gram + rod)

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15
Q

If you have a problem with your attack complex & can’t lyse holes into certain bacterial cell walls (C5-C9 problem)…what are you more susceptible to?

A

Neisseria

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16
Q

What does absence of C3 cause?

A

this is at the heart of the complement cascade
makes it difficult to respond to infections
get lots of pyogenic infections–can be confused w/ a phagocyte disorder

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17
Q

T/F To defeat neisseria, you need a fully functional complement system, especially the attack complex.

A

True.

18
Q

Why would a doctor be worried about recurrent otitis media?

A

if it is in a middle schooler or older person–>their eustachian tubes are no longer horizontal & they may have an immunodeficiency. Catch it now before they get pneumonia or something.

19
Q

What are some frequent organisms that bother people with B cell problems?

A

Moraxella
Haemophilus influenzae
Streptococcus pneumoniae
**the common things that bother kids

20
Q

What are some possible infections that people with B cell problems could get, aside from otitis media?

A

sinopulmonary problems

21
Q 
When you have infections from odd organisms, such as the following: Pnuemocystis carinii-known w/ AIDS 
Candida: invasive (lung/esophagus)
Systemic viral illness (CMV etc.)
Mycobacterial infections: systemic
What do you think might be the problem?
A

T cell problems!

22
Q

What are some important clinical features of T cell deficiencies?

A 
family hx of T cell problems
less than 6 mo
opportunistic infections
no lymph nodes
cutaneous lesions
increased chance of cancer
failure to thrive
GvH w/ mother's blood maybe
severe fungal or viral infection
fatality after BCG vaccine
diarrhea
hepatosplenomegaly
23
Q

What are some important clinical characteristics of B cell deficiencies?

A

family hx of B cell problems or of autoimmune or immunodeficiencies
onset after 6 mo when B cells are being produced
recurrent bacterial infections
allergy or autoimmune disease
vaccine failure
lots of sinopulmonary infections
perhaps a failure to thrive

24
Q

What are some important clinical characteristics of phagocytic deficiencies?

A

susceptible to low grade bacteria & fungi
severe infections, including pneumonia & osteomyelitis
skin infections
lymph adenitis
abscesses
delayed separation of the umbilical cord

25
Q

If you have a C3 complement deficiency…which types of infections will you start to see a lot?

A

recurrent bacterial infections

26
Q

If you have an attack complex (C5-C9) complement deficiency…which types of infections will you start to see a lot?

A

Neisseria infections

27
Q

What do azurophilic granules in neutrophils indicate?

A

Chediak Higashi Syndrome

**not picked up by a cytometer–have to look at a slide

28
Q

What does a bi-lobed nucleus indicate?

A

specific granule deficiency

29
Q

If you have neutropenia, what might you consider as potential causes?

A

congenital absence
autoantibody
cyclic neutropenia

30
Q

If you have thrombocytopenia & smaller platelets…which disorder will you start to consider?

A

WAS: Wiskott Aldrich Syndrome

31
Q

If you have RBC abnormalities…what are 2 disorders that you might anticipate?

A

autoimmune anemia

g6pd deficiency.

32
Q

If you see lymphopenia in an infant what might you consider?

A

SCID!! Yikes.

33
Q

What is the CH50 for?

A

a functional assay

if there is a deficiency anywhere in the complement system–>results will be zero.

34
Q

What is AH50 for?

A

a functional assay for the alternative pathway

if there is a deficiency somewhere in this–>results will be zero.

35
Q

IF both the CH50 & AH50 results come back as zero…what do we know?

A

There is a problem either in C3 or C5-C9. b/c these are the parts of the complement that they share.

36
Q

What would a low albumin tell you if you also had a low IgG?

A

It would tell you that perhaps the low IgG is due to a secondary cause, like diarrhea.

37
Q

If you suspect an immunodeficiency in a child but the immunology workup comes back normal…& there is nasal polyposis…what do you suspect?

A

cystic fibrosis

**also think about immotile cilia syndrome

38
Q

What are some protein vaccines?

A

diphtheria
tetanus
H. Influenza
**kids can deal with these vaccines.

39
Q

What are some polysaccharide vaccines?

A

pneumovax–can deal with this vaccine as an adult

40
Q

How long after a booster vaccine does it take before the pt starts making IgG?

A

1 mo

41
Q

If you suspect a T cell disorder…what are some labs that you might order?

A

HIV

CD3, CD4, CD8, CD19, CD56

42
Q

If FISH analysis shows a mutation in 22q11 what does that mean?

A

Di George

Block 6; Week 1-Michelle (6 decks)

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