section 22

Question 1

what diseases are considered to be MPNs

Answer 1

• CML (AP, BC)
• CNL
• chronic monocytic leukemia
• ET
• PMF
• PV
• eosinophilic leukemia

Question 2

common hematological features of CML

Answer 2

• shift left
• Philadelphia chromosome pos
• LAP decrease
• increased basophils
• hypercellular marrow

Question 3

describe the effects of Philadelphia chromosome

Answer 3

• causes a reciprocal translocation (9;22) that causes increased activity of tyrosine kinase
• tyrosine kinase causes uncontrolled proliferation of abnormal blood cells with lost apoptotic function

Question 4

hematologic features of CML-blast crisis

Answer 4

> 20% blasts with a shift to the left

Question 5

hematologic features of CNL

Answer 5

• WBC >80% PMNs and bands, no other immature grans
• toxic granulation and dohle bodies
• high LAP

Question 6

hematologic features of essential thrombocythemia (ET)

Answer 6

• increased BM megakaryopoeisis
• PLT aggregation and defects
• JAK-2 mutation

Question 7

hematologic features of primary myelofibrosis (PMF)

Answer 7

• marrow fibrosis w/ increased megakaryopoeisis
• early see increaed WBC
• progression = pancytopenia

Question 8

what are the 3 variations of polycythemia vera

Answer 8

1. polycythemia rubra vera
2. secondary polycythemia
3. relative erythrocytosis

Question 9

hematologic features of polycythemia rubra vera

Answer 9

• JAK-2 mutation
• hgb/hct increase
• normal arterial saturation
• increased LAP
• iron deficiency with progression

Question 10

what does the JAK-2 mutation effect in regards to red cells

Answer 10

causes red cell formation independent of EPO

Question 11

what are red cells formed independent of EPO called

Answer 11

endogenous erythroid colonies (EEC)

Question 12

characteristics of secondary polycythemia

Answer 12

increased red cell formation due to hypoxia
- low arterial saturation
- increased EPO
- normal LAP

Question 13

characteristics of relative erythrocytosis

Answer 13

• decrease in plasma vol increases red cell concentration
• elevated Hct
• normal red cell mass
• normal arterial saturation

Question 14

describe hematologic features that differ between CNL, CML and leukemoid rxn

Answer 14

• CNL: >80% bands and PMNs, high LAP
• CML: blasts with shift left (low LAP)
• leukemoid rxn: all stages of development present, high LAP

Question 15

define leukoerythroblastosis

Answer 15

the presence of immature red cells and a few immature white cells of the myeloid series in peripheral blood
- leuko = white
- erythro = red
- blastosis = increase of immature cells