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Block 6 Week 1 > Hogan: Primary Immunodeficiencies > Flashcards

Hogan: Primary Immunodeficiencies Flashcards

1
Q

What makes up about 1/2 of all primary immunodeficiencies?

A

antibody defects

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2
Q

Besides antibody defects, what is the next most commong cause of primary immunodeficiency disease?

A
  1. antibody defects
  2. combined immuno-deficiencies
  3. phagocytic defects
  4. T-cell defects
  5. complement defects
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3
Q

Is there complete phagocyte function in a neonate? What about complement function?

A

yes, leukocytes have passed from the liver and spleen to the bone marrow before birth and phagocyte functionality is complete; complement function is available in a neonate due to maternal passage of IgG, however, antibody mediated complement fixation lags until Ab production is initiated in a neonate (~6mo)

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4
Q

Is NK cell function available in neonates?

A

yes

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5
Q

Is T cell function complete at birth?

A

yes, via thymic development

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6
Q

Is B cell function complete at birth?

A

no, B cells must wait for T cells to mature before they can mature and have functional antibody function; T cells are functional at birth, but B cells are not functional until about 6-24 months.

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7
Q

What happens to B cells if T cells fail to develop?

A

B cell functionality will suffer

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8
Q

For approximately how many months of a neonate’s life is he/she protected by maternal antibodies?

A

6 months; after this, the 1/2 life is about over and the child can begin to develop his/her own antibodies.

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9
Q

What comes first - immunodeficiency or autoimmunity?

A

Either/or!

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10
Q

What do autoimmune problems in a patient suggest difficulty in?

A

B cell maturation
isotype switching
tolerance

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11
Q

Phagocytes are (blank): Both Gram+ and Gram- and yeast/fungal organisms are attacked

A

non-discriminatory

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12
Q

What are these types of organisms typically associated with:

Staphylococci aureus
Pseudomonas aeroginosa
Aspergillus fumigatus (fungal)
Candida (yeast)
Enterbacteriaceae
Others:  Nocardia (Gram + rod) /Listeria (Gram +  rod)
A

neutrophil disorders

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13
Q

Failure of the attack complex in complement disorders result in susceptibility to (blank)

A

Neisseria

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14
Q

Absence of (blank), which is at the heart of the complement cascade, makes the response to severe bacterial infections difficult

A

C3

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15
Q

Otitis media, draining ears, sinusitis, pulmonary infections are common in what type of deficiency?

A

B cell (humoral) deficiency

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16
Q

Why are recurrent ear infections (otitis media) abnormal after children reach middle school age?

A

eustachian tube anatomy improves with facial growth to allow for “downhill” drainage

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17
Q

What are these organism likely associated with?

Pnuemocystis carinii
Candida: invasive (lung/esophagus)
Systemic viral illness (CMV etc.)
Mycobacterial infections

A

T cell problems

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18
Q

What are some clinical characteristics of T cell deficiencies?

A 
family history
onset BEFORE 6mo
failure to thrive
no lymph nodes
cutaneous lesions
severe fungal/viral infection
diarrhea
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19
Q

What are some clinical characteristics of B cell deficiencies?

A 
family history of autoimmune/immunodeficiency
recurrent virulent bacterial infections
allergy/autoimmune disease
vaccine failure
sinopulmonary infections
FTT
20
Q

What are some clinical characteristics of phagocytic deficiencies?

A 
susceptible to low grade bacteria and fungus
severe infections
skin infections
lymphadenitis
abscesses
delayed umbilical cord separation
21
Q

If you have a C3 complement deficiency, how might you present?

A

with recurrent bacterial infections

22
Q

If you have deficiencies in C5-C8 complement proteins, what might you present with?

A

Neisseria infections

23
Q

What is the best lab test to order if you suspect an immunodeficiency?

A

CBC with differential

24
Q

If you have azurophilic granules, what do you think of?

A

Chediak Higashi

25
Q

If you have increased neutrophils, what do you think of?

A

infection

leukocyte adhesion deficiency (cannot diapedis)

26
Q

If you have decreased neutrophils, what do you think of?

A

congenital absence
autoantibody
cyclic neutropenia

27
Q

With RBC abnormalities, what do you consider?

A

autoimmune anemia

g6pd deficiency

28
Q

If low lymphocytes (lymphopenia), what do you consider?

A

SCID

29
Q

If you have small platelets or a decreased number, what do you think of?

A

Wiskott-Aldrich syndrome

30
Q

If you have ZERO CH50, what is wrong?

A

a genetic defect in the complement cascade **order individual complement components to sort it out
profound sepsis

31
Q

If you have ZERO AH50, what is wrong?

A

genetic deficiency in the complement cascade

32
Q

If AH50 and CH50 are BOTH zero, which complement proteins might be impaired?

A

C3, C5-C9

**test specific complement components in this case

33
Q

When measuring IgA, what should you keep in mind?

A

any value greater than zero is OK despite what reference values predict

34
Q

Why is albumin testing relevant to B cell lab work?

A

it allows you to determine if IgG loss is secondary

35
Q

If you have a child with nasal polyps, what should you think of?

A

cystic fibrosis

36
Q

The following vaccines have what component?

diphtheria
tetanus
H flu

A

protein component

37
Q

What is an isohemagluttinin?

A

IgM molecule that helps identify which ABO blood group a person belongs to

38
Q

How long will it take to make IgG after a booster shot?

A

1 month

39
Q

What are CD3, CD4, and CD8 markers for?

A

T cells

40
Q

What is CD19 a marker for?

A

B cells

41
Q

What are some suspected T cell disorders?

A

HIV

deficient in CD3, CD4, CD8, 19 or 56

42
Q

When is it appropriate to do specific antigen studies?

A

if a patient is >1yo and/or has been vaccinated

43
Q

What can be used to stimulate lymphocytes?

A

mitogens

44
Q

In a neonatal chest X ray, what should you look for?

A

“sail sign” - the thymus

45
Q

What test can be used to determine neutrophil functionality?

A

flow cytometry

46
Q

What are some red flags for immunodeficiency in regards to pneumonia?

A

> 2 cases of pneumonia

pneumonia that requires hospitalization

Block 6 Week 1 (13 decks)

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