PV facts

Question 1

Median age PV

Answer 1

61

Question 2

Incidence PV

Answer 2

2/100K

Question 3

JAK2 VAF >50% clinical risk in PV

Answer 3

Thrombosis
Splenomegaly
Progression to PMF
Leukocytosis
Higher Hb

Question 4

MIPSS-PV factors

Answer 4

Age
Thrombosis
WBC
SRSF2

Assesses the risk for MF or AML not a sole criteria for Tx intiation

Question 5

2nd line Tx for PV

Answer 5

Ruxo (RESPONSE- phase III vs SOC, better control of Hb, spleen, and Sx)
PEG-IFN

Question 6

3rd line Tx for PV

Answer 6

Busulfan

Question 7

ROPEG-IFN vs HU in PV 1st line

Answer 7

PROUD/Continuation-PV
Phase III
vs HU

PEG was:
- More effective in controlling Hb, VAF reduction, and reducing fibrosis
- Equally effective in preventing thrombosis
- Safe
- Slower acting

Control arm in PROUD was already HU resistant, and Tx with HU

Question 8

Rusfertide in PV

Answer 8

Hepcidin mimetic
May lower Hb without phlebotomy
less iron def Sx

Question 9

LDH in PV vs ET

Answer 9

LDH may be elevated in PV but not commonly elevated in ET, alarm bells for PMF

Question 10

PV diagnosis

Answer 10

JAK2
+
Hb>16,16.5
+
BM panmyelosis without dyplasia or EPO< LLN

Question 11

PV risk transform to MF

Answer 11

20%