Learning Objectives

  • Review the available data regarding choice and duration of anticoagulation to prevent recurrent thrombosis in polycythemia vera

  • Discuss the available data regarding cytoreductive agents and their efficacy for secondary thrombosis prevention in polycythemia vera

A 50-year-old woman develops a right femoral deep vein thrombosis. Laboratory results reveal a hemoglobin of 17  g/dL, hematocrit of 50%, platelet count 560 K/µL, and erythropoietin level of 1.5 IU/L. Molecular testing reveals a JAK2 V617F mutation with a variant allele frequency (VAF) of 60%. Additional hypercoagulable workup is negative.

Polycythemia vera (PV), one of the myeloproliferative neoplasms (MPNs), is a clonal hematologic malignancy characterized by activating mutations in JAK2. Approximately 95% of patients with PV have mutations in either Janus kinase 2 (JAK2) V617F or exon 12 resulting in excess erythropoiesis, increased red blood cell mass, and aberrant cytokine...

1.
Kelliher
S
,
Falanga
A
.
Thrombosis in myeloproliferative neoplasms: a clinical and pathophysiological perspective
.
Thromb Update
.
2021
;
5
(
6
):
100081
.
2.
Hultcrantz
M
,
Björkholm
M
,
Dickman
PW
, et al.
Risk for arterial and venous thrombosis in patients with myeloproliferative neoplasms: a population-based cohort study
.
Ann Intern Med
.
2018
;
168
(
5
):
317
-
325
.
3.
Panova-Noeva
M
,
Marchetti
M
,
Russo
L
, et al.
ADP-induced platelet aggregation and thrombin generation are increased in essential thrombocythemia and polycythemia vera
.
Thromb Res
.
2013
;
132
(
1
):
88
-
93
.
4.
De Stefano
V
,
Za
T
,
Rossi
E
, et al
;
GIMEMA CMD-Working Party
.
Recurrent thrombosis in patients with polycythemia vera and essential thrombocythemia: incidence, risk factors, and effect of treatments
.
Haematologica
.
2008
;
93
(
3
):
372
-
380
.
5.
Barbui
T
,
Finazzi
G
,
Falanga
A
.
Myeloproliferative neoplasms and thrombosis
.
Blood
.
2013
;
122
(
13
):
2176
-
2184
.
6.
Gerds
AT
,
Mesa
R
,
Burke
JM
, et al.
Association between elevated white blood cell counts and thrombotic events in polycythemia vera: analysis from REVEAL
.
Blood
.
2024
;
143
(
16
):
1646
-
1655
.
7.
Carobbio
A
,
Ferrari
A
,
Masciulli
A
,
Ghirardi
A
,
Barosi
G
,
Barbui
T
.
Leukocytosis and thrombosis in essential thrombocythemia and polycythemia vera: a systematic review and meta-analysis
.
Blood Adv
.
2019
;
3
(
11
):
1729
-
1737
.
8.
Moliterno
AR
,
Kaizer
H
,
Reeves
BN
.
JAK2 V617F allele burden in polycythemia vera: burden of proof
.
Blood
.
2023
;
141
(
16
):
1934
-
1942
.
9.
De Stefano
V
,
Ruggeri
M
,
Cervantes
F
, et al.
High rate of recurrent venous thromboembolism in patients with myeloproliferative neoplasms and effect of prophylaxis with vitamin K antagonists
.
Leukemia
.
2016
;
30
(
10
):
2032
-
2038
.
10.
Hernández-Boluda
J-C
,
Arellano-Rodrigo
E
,
Cervantes
F
, et al
;
Grupo Español de Enfermedades Mieloproliferativas Filadelfia Negativas (GEMFIN)
.
Oral anticoagulation to prevent thrombosis recurrence in polycythemia vera and essential thrombocythemia
.
Ann Hematol
.
2015
;
94
(
6
):
911
-
918
.
11.
Wang
T-F
,
Khorana
A-A
,
Agnelli
G
, et al.
Treatment of cancer-associated thrombosis: recent advances, unmet needs, and future direction
.
Oncologist
.
2023
;
28
(
7
):
555
-
564
.
12.
Hamulyák
EN
,
Daams
JG
,
Leebeek
FWG
, et al.
A systematic review of antithrombotic treatment of venous thromboembolism in patients with myeloproliferative neoplasms
.
Blood Adv
.
2021
;
5
(
1
):
113
-
121
.
13.
Huenerbein
K
,
Sadjadian
P
,
Becker
T
, et al.
Direct oral anticoagulants (DOAC) for prevention of recurrent arterial or venous thromboembolic events (ATE/VTE) in myeloproliferative neoplasms
.
Ann Hematol
.
2021
;
100
(
8
):
2015
-
2022
.
14.
Fedorov
K
,
Goel
S
,
Kushnir
M
,
Billett
HH
.
Thrombosis in myeloproliferative neoplasms: treatment outcomes of direct oral anticoagulants and vitamin K antagonists
.
Res Pract Thromb Haemost
.
2021
;
5
(
6
):
e12574
.
15.
Landolfi
R
,
Marchioli
R
,
Kutti
J
, et al
;
European Collaboration on Low-Dose Aspirin in Polycythemia Vera Investigators
.
Efficacy and safety of low-dose aspirin in polycythemia vera
.
N Engl J Med
.
2004
;
350
(
2
):
114
-
124
.
16.
De Stefano
V
,
Finazzi
G
,
Barbui
T
.
Antithrombotic therapy for venous thromboembolism in myeloproliferative neoplasms
.
Blood Cancer J
.
2018
;
8
(
7
):
65
.
17.
Marchioli
R
,
Finazzi
G
,
Specchia
G
,
Masciulli
A
,
Mennitto
MR
,
Barbui
T
.
The CYTO-PV: a large-scale trial testing the intensity of CYTOreductive therapy to prevent cardiovascular events in patients with polycythemia vera
.
Thrombosis
.
2011
;
2011
:
794240
.
18.
Barbui
T
,
De Stefano
V
,
Ghirardi
A
,
Masciulli
A
,
Finazzi
G
,
Vannucchi
AM
.
Different effect of hydroxyurea and phlebotomy on prevention of arterial and venous thrombosis in polycythemia vera
.
Blood Cancer J
.
2018
;
8
(
12
):
124
.
19.
Vannucchi
AM
,
Kiladjian
JJ
,
Griesshammer
M
, et al.
Ruxolitinib versus standard therapy for the treatment of polycythemia vera
.
N Engl J Med
.
2015
;
372
(
5
):
426
-
435
.
20.
Harrison
CN
,
Nangalia
J
,
Boucher
R
, et al.
Ruxolitinib versus best available therapy for polycythemia vera intolerant or resistant to hydroxycarbamide in a randomized trial
.
J Clin Oncol
.
2023
;
41
(
19
):
3534
-
3544
.
21.
Gisslinger
H
,
Klade
C
,
Georgiev
P
, et al
;
PROUD-PV Study Group
.
Ropeginterferon alfa-2b versus standard therapy for polycythaemia vera (PROUD-PV and CONTINUATION-PV): a randomised, non-inferiority, phase 3 trial and its extension study
.
Lancet Haematol
.
2020
;
7
(
3
):
e196
-
e208
.
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