Week 1

Question 1

What type of anemia is associated with vitamin B6 deficiency?

Answer 1

microcytic, hypochromic (due to slowed heme production as delta-ALA synthase requires pyroxidal phosphate)

Question 2

What are porphyrias?

Answer 2

inherited disorders resulting from enzyme deficiencies in the heme biosynthesis pathway

Question 3

What enzyme deficiency is associated with sideroblastic anemia?

Answer 3

ALA synthase deficiency

Question 4

If you suspect a porphyria but urine PBG is negative, which enzyme may be deficient?

Answer 4

-ALA dehydratase

Question 5

What is the enzyme deficiency in Acute Intermittent Porphyria?

Answer 5

-PBG deaminase

Question 6

What are 2 lab values to differentiate Anemia of Chronic Disease from Iron Deficiency Anemia?

Answer 6

AOCD: reduced/normal transferrin; normal/ increased ferritin
IDA: increased transferrin; reduced ferritin

Question 7

What gene is associated with type 1 Hereditary Hemochromatosis?

Answer 7

• C282Y, cysteine to tyrosine substitution at AA 282

- deficiency of hepcidin expression causes increased iron absorption

Question 8

What is oncotic pressure? What is hydrostatic pressure?

Answer 8

• oncotic pressure: draw fluid back into capillaries at venous end from proteins in blood (ex: albumin)
• hydrostatic pressure: causes water to leave capillaries, P falls at venous end

Question 9

What is the difference between a reticulocyte and a mature RBC? Where does it finish maturation?

Answer 9

• reticulocytes retain ribosomes and mRNA, capable of synthesizing hemoglobin
• mature in spleen

Question 10

To bind oxygen, does iron of hemoglobin need to be in ferrous (+2) or ferric (+3) state?

Answer 10

• ferrous (+2)

- ROS can oxidize the iron to ferric (+3) state = methemoglobin

Question 11

What is a CFU (colony forming unit)?

Answer 11

• an undifferentiated pluripotent stem cell that originates from mesenchymal cells
• CFU seeds liver, spleen, and bone marrow

Question 12

What are the divisions of the lymph node cortex? What cells are found at each division?

Answer 12

• Superficial cortex (B-lymphocytes)
• Mid-cortex (germinal centers, majority B-lymphocytes)
• Deep cortex (between germinal centers and medullary cords, majority T-lymphocytes)

Question 13

What types of cells are found in the lymph node sinuses?

Answer 13

macrophages, lymphocytes, and reticular cells

Question 14

What kind of endothelium lines the outer cortex of blood flow through lymph node? deep cortex?

Answer 14

• outer cortex: lined by simple squamous endothelium

- deep cortex: lined by simple cuboidal endothelium (HEV’s= high endothelial venules)

Question 15

EBV protein LMP-1 activates what transcription factors?

Answer 15

NF-kappaB

-LMP-1 mimics host cell’s CD40

Question 16

EBV protein LMP-2 activates what transcription factors?

Answer 16

• fos/jun (et al)

- LMP2 mimics host cell’s B receptor (BCR) and promotes MAPK activation and transcription of fos/jun regulated genes

Question 17

What are the “classic triad” symptoms of infectious mono?

Answer 17

• pharyngitis
• lymphadenopathy
• fever