Polycythemia Vera

Question 1

Median survival of PV patients

Answer 1

14 years

Question 2

Possible transformations of PV

Answer 2

Myelofibrosis

AML

Question 3

what characterizes PV pathophysiologically?

Answer 3

Growth factor (epo) independent proliferation of erythroid cells

Question 4

Treatment

Answer 4

Phlebotomy
Goal hematocrit 45%
Hydroxyurea
IF refractory → Jakafi
Aspirin 81 mg daily
IF thrombocythemia → anagrelide
B12 + LAP

Question 5

Hct goal of treatment

Answer 5

hematocrit 45%

Question 6

why do you give hydrea?

Answer 6

• Reduces RBC and platelet counts in order to reduce risk of thrombotic events

Question 7

jakafi generic name

Answer 7

Ruxolitinib

Question 8

Diagnosis + specifications

Answer 8

WHO criteria (must meet all 3 major criteria or first 2 major criteria and minor criteria)

Question 9

Major criteria in WHO classification

Answer 9

1) Hgb greater than 16.5 in men or 16 in women, OR HCT greater than 49% in men or greater than 48% in women
2) BMB showing hypercellularity with trilineage growth (prominent erythroid, granulocytic, and megakaryocytic proliferation with pleomorphic, mature megakaryocytes)
3) Presence of JAK2 V617F or JAK2 exon 12 mutation
MINOR
1) Subnormal epo level

Question 10

Goal hematocrit with phlebotomy

Answer 10

45%

Question 11

Bone marrow findings in PV

Answer 11

hypercellularity with trilineage growth (prominent erythroid, granulocytic, and megakaryocytic proliferation with pleomorphic, mature megakaryocytes)