Thrush- Immunological pathologies II

Question 1

what is a primary immunodeficiency?

Answer 1

an inherited defect

Question 2

what is a secondary immunodeficiency?

Answer 2

acquired defect (infections, aging, malignancies and therapy)

Question 3

does immunodeficiency lead to both lymphoid and myeloid deficiencies?

Answer 3

yes

Question 4

what are common features of severe combined immunodeficiencies (SCID)?

Answer 4

have defect in T-cell compartment, low number of circulating lymphocytes, severe recurrent infections, myeloid and erythroid lineages are normal

Question 5

how would you treat a SCID?

Answer 5

bone marrow transplant (can prolong life in sterile environment until treatment is available)

Question 6

what happens in X-linked IL-2Rgamma chain deficiency?

Answer 6

the gamma chain (signaling chain) is prevented from activating Th1 and Th2

Question 7

what happens in JAK-3 deficiency?

Answer 7

JAK defect will lead to the improper cytokine signaling

Question 8

could a T cell be theoretically activated if there is a defect in the alpha chain of the IL-2 receptor of CD25?

Answer 8

yes, because the beta subunit can still weakly bind and allow the gamma chain to carry out signaling

Question 9

what do defects in RAG1/RAG2, alpha-chain of IL-2 receptor, and adenosine deaminase have in common?

Answer 9

they are all autosomal mutants

Question 10

does a RAG mutation only lead to a lack of B cells?

Answer 10

nope. both T and B cells

Question 11

what disease contains a defect in MHC class II transactivator protein gene (lack of MHC II)?

Answer 11

bare lymphocyte syndrome

Question 12

what is a TAP gene defect?

Answer 12

lack of MHC class I

Question 13

what does X-linked hypogammaglobulinemia an example of? and what does it inhibit?

Answer 13

B cell defect.

unable to rearrange light chain, so patient will lack peripheral B cells

Question 14

what does a defect in CD40L on T cells lead too?

Answer 14

prevents B cells from class switching from IgM to IgG and IgA

Question 15

IgA is the most common deficiency, what would this lead too?

Answer 15

recurrent respiratory infections

Question 16

what myeloid immunodefeciency, leads to a defect in oxidative burst and also leads to negative Nitroblue tetrazolium dye (NBT) test?

Answer 16

chronic gramulomatous disease (CGD)

Question 17

what secondary immunodeficiency primarily infects CD4+ T cells?

Answer 17

HIV/AIDS

Question 18

are lymphoid cancer patients often immunosuppressed?

Answer 18

yes

Question 19

why does cancer therapy often cause immunosuppression?

Answer 19

many chemotherapy drugs and radiation targets rapidly dividing cells (bone marrow especially)

Question 20

when would an individual experience loss of filtration and phagocytic cells (leading to increased bacterial infections)?

Answer 20

splenectomy

Question 21

what type of immunodeficiency therapy occurs when stem cells are being replaced?

Answer 21

BMT