Immunodeficiencies - Mandal

Question 1

Explain the difference between congenital (primary) and acquired (secondary) immunodeficiencies are

Answer 1

Primary (congenital)- born with it

Secondary (acquired)- got later

Question 2

What is Hypogammaaglobulinemia of Infancy?

Answer 2

Humoral

delay in B cell development after weaned of maternal IgG resulting in Ab deficiency

Question 3

What is Bruton’s Agammaglobulinemia?

Answer 3

Humoral
Failure of B cells to mature in the bone marrow
Need to use vaccination (IVIg) (cannot use live atteuated)
For infections, need heavy antibiotics

Question 4

What is Hyper-IgM Syndrome?

Answer 4

Humoral
B cells can’t go class switching (isotope switching)
Treat with IVIg vaccination

Question 5

What is IgA deficiency?

Answer 5

Humoral

low serum IgA, linked with celiac disease

Question 6

What is Common Variable Immunodeficiency (CVID)?

Answer 6

Humoral
Absence of plasma cells
Treat with IVIg vaccination

Question 7

What is DiGeorge Syndrome?

Answer 7

DIE GEORGE DIE WITH YOUR TINY THYMUS
Cellular
T cells absent or greatly reduced, which results in recurrent viral infections
Treat: transplant of fetal thymus

Question 8

What is Severe Combined Immunodeficiency (SCID)?

Answer 8

“Boy in the Plastic Bubble”
Combined
greatly reduced T and B cells
Treat: bone marrow transplant

Question 9

Chronic Granulomatous Disease (CGD)?

Answer 9

Combined
Failure of phagocytes to completely clear bacteria due to defects in NADPH oxidase
Treat: long term antibiotics, injections of IFN-gamma to activate macrophages

Question 10

How does HIV work?

Answer 10

HIV infects CD4 which present to CD8 which kill the CD4. Reduces CD4 greatly until you are immunosuppressed

Question 11

What are the first signs of HIV?

Answer 11

oral candidiasis