Immunodeficiencies B-Cell Disorders

Question 1

MC Immunoglobulin def.?

Answer 1

Selective IgA

Question 2

With Selective IgA what symptoms do you get?

Answer 2

Recurrent Otitis Media

Question 3

Specific symptom of selective IgA (think Blood)?

Answer 3

If you transfuse RBCs, transfuse IgA=> Anaphylatic reaction=>patient dies and has intense rash

Question 4

What labs do you see in Selective IgA def.?

Answer 4

Decrased IgA

Question 5

What disease is associated with Selective IgA?

Answer 5

Celiac Disease

Question 6

Xlinked (bruton) agammaglobulinemia MOA?

Answer 6

Defect in BTK, a tyrosine kinase gene=>􏰀no B-cell maturation. X-linked recessive

Question 7

Bruton is seen more in?

Answer 7

Boys

Question 8

CVID MOA?

Answer 8

Defect in B-cell differentiation

Question 9

CVID increases your risk for?

Answer 9

autoimmune disease and lymphoma

Question 10

In Bruton’s Agammaglobinemia, are all IgG decreased?

Answer 10

Yes

Question 11

Bruton’s patients get recurrent infection of ?

Answer 11

Giardia