04b: Heme 2

Question 1

Polycythemia vera Rx:

Answer 1

Phlebotomy, hydroxyurea, JAK inhibitor (ruxolitinib)

Question 2

The (X) mutation in the myeloproliferative disorders makes hematopoietic cells (more/less) sensitive to:

Answer 2

X = JAK2 (V607F)

More; growth factors (EPO, TPO)

Question 3

Polycythemia vera: (increased/decreased) RBCs, (inc/dec) WBCs, (inc/dec) platelets.

Answer 3

All increased

Question 4

T/F: RBCs are decreased in myelofibrosis.

Answer 4

True

Question 5

Inappropriate absolute polycythemia: (high/low) EPO, (high/low) RBC mass, (high/low) plasma volume. What is a cause of this?

Answer 5

High, high; no change in plasma V

Ectopic EPO (malignancy)

Question 6

Occasional involvement of t(9;22) in (X) leukemia confers poor prognosis than if translocation wasn’t present.

Answer 6

X = ALL

Question 7

Dipyridamole and cilostazol are drugs that (stimulate/inhibit) (X) and can be very useful for (Y).

Answer 7

Inhibit
X = PDE (increase cAMP; inhibit platelet aggregation and stim arteriolar vasodilation)
Y = intermittent claudication

“Let’s build cAMP in DA PYRAMIDs as high as el CIELO”

Question 8

Which lymph node structure will be underdeveloped in DiGeorge Syndrome?

Answer 8

Paracortex (location of T-lymphocytes)

Question 9

Cancer immunotherapy involves anti-(X) Ab that block receptors on (Y) cells and keep them activated.

Answer 9

X = PD1 ("programmed death R 1" or CTLA4 ("cytotoxic T Lymphocyte associated protein 4")
Y = T-lymphocytes

Question 10

Antibody binding CD28 on (X) immune cells would (increase/decrease) activation.

Answer 10

X = T-lymphocytes

Decrease (decrease interaction with B7 on APCs, which is a co-stimulatory signal for T cell activation)

Question 11

Which immune cells are “atypical” in EBV infection?

Answer 11

CD8+ T-lymphocytes

Question 12

1 yo child with eczema, thrombocytopenia, and recurrent severe respiratory infections.

Answer 12

Wiskott-Aldrich Syndrome (X-linked R; combined B and T lymphocyte disorder)

Question 13

Where does B cell isotype switching occur? Interaction between which R and ligand is necessary to instigate this switch?

Answer 13

Germinal follicles (in lymph node)

CD40 (B cell) and CD40L (active T cell)

**NOTE importance of T cell in this role! This is why AIDs patients can only produce IgM (which doesn’t require isotype switching/T cells)

Question 14

(Positive/negative) selection involves thymic cortical epithelial cells. (Positive/negative) selection involves thymic medullary epithelial cells and (X) cells.

Answer 14

Positive (in cortex)

Negative (in medulla)
X = dendritic

Question 15

Baby born with hydrops fetalis, anemia, jaundice, extramedullary hematopoeisis (hepatosplenomegaly), and positive Coombs test.

Answer 15

Hemolytic disease of newborn (mom’s Ab attacked)

Question 16

“-tinib” drugs (stimulate/inhibit):

Answer 16

Inhibit

Tyr kinase

Question 17

Vemurafenib MOA:

Answer 17

BRAF inhibitor (metastatic melanoma)

VEmuRAF-enib = V600E-MUtated bRAF inhibition

Question 18

Tumor lysis syndrome: which electrolyte and metabolic abnormalities are of great concern?

Answer 18

1. Hyperkalemia and hyperphosphatemia (released from lysed cells)
2. Hypocalcemia (bound by PO4)
3. Hyperuricemia (nucleic acid breakdown)

Question 19

Rx protocol for tumor lysis syndrome

Answer 19

1. Aggressive hydration

2. Allopurinol and rasburicase