Allergy/Immuno

Question 1

CVID is due to?
How does it present?
What is there an increased risk of?
How do you diagnose?
How do you treat?

Answer 1

B cells do not make effective amounts of Igs
Recurrent sinopulm infections in adults
Lymphoma
Decreased Ig levels + decreased response to antigen stim of B cells
Abx acutely + regular IVIG

Question 2

What is XL agammglobulinemia?
What is affected?
How do you treat?

Answer 2

B and lymphoid cells are diminished
Increased pulm infections
IVIG

Question 3

How do you treat SCID?

Answer 3

BM transplant is curative!

Question 4

What is unique about IgA deficiency?

How do you treat?

Answer 4

Anaphylaxis to blood transfusions if there is normal IgA levels
No need - but avoid IgA in blood transfusions!

Question 5

How does Hyper IgE present?

What can be used for prophylaxis?

Answer 5

Recurrent Staph skin infections

Dicloxacillin, Cephalexin

Question 6

How does WAS present?

What is the treatment?

Answer 6

Thrombocytopenia + eczema + T cell deficiency

BM transplant

Question 7

How does CGD present?
What are 4 bugs that are clues?
How do you diagnose?

Answer 7

Lymph nodes with purulent material leaking out
Staph + Burkholderia + Nocardia + Aspergillus
Nitroblue Tetrazolium: detects decrease in respiratory burst

Question 8

What are 3 causes of angioedema?
What is absent in angioedema?
What does not hereditary angioedema not respond to?
What is the best initial test?
What is the acute therapy?
What is long term therapy?

Answer 8

Idiopathic, C1 esterase deficiency, ACEi initiation
Pruritus/urticaria
Steroids
Decreased C2 and C4
Airway protection + Ecallantide, FFP
Androgens