Polycythemia Vera Flashcards Preview

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Flashcards in Polycythemia Vera Deck (11)
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1
Q

Median survival of PV patients

A

14 years

2
Q

Possible transformations of PV

A

Myelofibrosis

AML

3
Q

what characterizes PV pathophysiologically?

A

Growth factor (epo) independent proliferation of erythroid cells

4
Q

Treatment

A
Phlebotomy
Goal hematocrit 45%
Hydroxyurea
IF refractory → Jakafi
Aspirin 81 mg daily
IF thrombocythemia → anagrelide
B12 + LAP
5
Q

Hct goal of treatment

A

hematocrit 45%

6
Q

why do you give hydrea?

A
  • Reduces RBC and platelet counts in order to reduce risk of thrombotic events
7
Q

jakafi generic name

A

Ruxolitinib

8
Q

Diagnosis + specifications

A

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

9
Q

Major criteria in WHO classification

A

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

10
Q

Goal hematocrit with phlebotomy

A

45%

11
Q

Bone marrow findings in PV

A

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

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