Abstract
The BCR-ABL-negative myeloproliferative neoplasms (MPN) are associated with high incidence of venous thrombosis and a significant rate of recurrent events, but there is no consensus regarding their management. In this retrospective study, we analyzed 96 patients with MPN-related venous thrombosis. The index venous thrombosis occurred at a median age of 58 years (IQR 37–71), with 58% of the events involving unusual sites. Patients who were on antiplatelet agents at the time of index thrombosis tended to be older than patients who were not receiving antiplatelets at the time of index thrombosis. The majority of index thromboses occurring after the diagnosis of MPN had uncontrolled blood counts at the time of event and were not receiving antithrombotic agents. Following the thrombotic episode, 75% of patients received long-term anticoagulation. At a median follow-up of 3.4 years, the recurrence rate was 14%. Thrombophilia was significantly more prevalent among patients with recurrent thrombosis compared to patients without recurrence (p < 0.01). Patients who developed a recurrent event early were more likely to have thrombophilia (either inherited or antiphospholipid antibodies), and controlled blood counts, and were likely to receive anticoagulation at the time of recurrence compared to patients with later recurrences. Thrombophilia may contribute to venous thrombosis recurrence, especially early after the index venous thrombosis. Suboptimal anticoagulation and blood count control are factors associated with late venous thrombosis recurrence.
References
1.
Ball
S
, Thein
KZ
, Maiti
A
, Nugent
K
. Thrombosis in Philadelphia negative classical myeloproliferative neoplasms: a narrative review on epidemiology, risk assessment, and pathophysiologic mechanisms
. J Thromb Thrombolysis
. 2018
;45
(4
):516
–28
. 2.
Smalberg
JH
, Arends
LR
, Valla
DC
, Kiladjian
J-J
, Janssen
HL
, Leebeek
FW
. Myeloproliferative neoplasms in Budd-Chiari syndrome and portal vein thrombosis: a meta-analysis
. Blood
. 2012
;120
(25
):4921
–8
.3.
Martinelli
I
, De Stefano
V
, Carobbio
A
, Randi
ML
, Santarossa
C
, Rambaldi
A
, et al Cerebral vein thrombosis in patients with Philadelphia-negative myeloproliferative neoplasms. An European Leukemia Net study
. Am J Hematol
. 2014
;89
(11
):E200
–5
. .4.
Akpan
IJ
, Stein
BL
. Splanchnic vein thrombosis in the myeloproliferative neoplasms
. Curr Hematol Malig Rep
. 2018
;13
(3
):183
–90
. .5.
Hultcrantz
M
, Bjӧrkholm
M
, Dickman
PW
, Landgren
O
, Derolf
ÅR
, Kristinsson
SY
, 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
–25
.6.
Landolfi
R
, Di Gennaro
L
, Barbui
T
, De Stefano
V
, Finazzi
G
, Marfisi
R
, et al Leukocytosis as a major thrombotic risk factor in patients with polycythemia vera
. Blood
. 2007
;109
(6
):2446
–52
. .7.
De Stefano
V
, Za
T
, Rossi
E
, Vannucchi
AM
, Ruggeri
M
, Elli
E
, et al Recurrent thrombosis in patients with polycythemia vera and essential thrombocythemia: incidence, risk factors, and effect of treatments
. Haematologica
. 2008
;93
(3
):372
–80
. .8.
Elliott
MA
, Pardanani
A
, Lasho
TL
, Schwager
SM
, Tefferi
A
. Thrombosis in myelofibrosis: prior thrombosis is the only predictive factor and most venous events are provoked
. Haematologica
. 2010
;95
(10
):1788
–91
. .9.
Hernández-Boluda
JC
, Arellano-Rodrigo
E
, Cervantes
F
, Alvarez-Larrán
A
, Gómez
M
, Barba
P
, et al Oral anticoagulation to prevent thrombosis recurrence in polycythemia vera and essential thrombocythemia
. Ann Hematol
. 2015
;94
(6
):911
–8
. .10.
De Stefano
V
, Ruggeri
M
, Cervantes
F
, Alvarez-Larrán
A
, Iurlo
A
, Randi
M
, 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
–38
.11.
Landolfi
R
, Marchioli
R
, Kutti
J
, Gisslinger
H
, Tognoni
G
, Patrono
C
, et al Efficacy and safety of low-dose aspirin in polycythemia vera
. N Engl J Med
. 2004
;350
(2
):114
–24
.12.
Kreher
S
, Ochsenreither
S
, Trappe
RU
, Pabinger
I
, Bergmann
F
, Petrides
PE
, et al Prophylaxis and management of venous thromboembolism in patients with myeloproliferative neoplasms: consensus statement of the Haemostasis Working Party of the German Society of Hematology and Oncology (DGHO), the Austrian Society of Hematology and Oncology (ÖGHO) and Society of Thrombosis and Haemostasis Research (GTH eV)
. Ann Hematol
. 2014
;93
(12
):1953
–63
.13.
Barbui
T
, Tefferi
A
, Vannucchi
AM
, Passamonti
F
, Silver
RT
, Hoffman
R
, et al Philadelphia chromosome-negative classical myeloproliferative neoplasms: revised management recommendations from European LeukemiaNet
. Leukemia
. 2018 May
;35
(5
):1057
–69
.14.
Network NCC, others. NCCN clinical practice guidelines in oncology. Myeloproliferative neoplasms. Version 2.2018. 2018
.15.
De Stefano
V
, Rossi
E
, Carobbio
A
, Ghirardi
A
, Betti
S
, Finazzi
G
, et al Hydroxyurea prevents arterial and late venous thrombotic recurrences in patients with myeloproliferative neoplasms but fails in the splanchnic venous district. Pooled analysis of 1500 cases
. Blood Cancer J
. 2018
;8
(11
):65
. .16.
Ellis
MH
, Lavi
N
, Vannucchi
A
, Harrison
C
. Treatment of thromboembolic events coincident with the diagnosis of myeloproliferative neoplasms: a physician survey
. Thromb Res
. 2014
;134
(2
):251
–4
. .17.
Wille
K
, Sadjadian
P
, Becker
T
, Kolatzki
V
, Horstmann
A
, Fuchs
C
, et al High risk of recurrent venous thromboembolism in BCR-ABL-negative myeloproliferative neoplasms after termination of anticoagulation
. Ann Hematol
. 2019
;98
(1
):93
–100
. .18.
Le Calloch
R
, Lacut
K
, Le Gall-Ianotto
C
, Nowak
E
, Abiven
M
, Tempescul
A
, et al Non-adherence to treatment with cytoreductive and/or antithrombotic drugs is frequent and associated with an increased risk of complications in patients with polycythemia vera or essential thrombocythemia (OUEST study)
. Haematologica
. 2018
;103
(4
):607
–13
. .19.
Harrison
C
, Donohoe
S
, Carr
P
, Dave
M
, Mackie
I
, Machin
S
. Patients with essential thrombocythaemia have an increased prevalence of antiphospholipid antibodies which may be associated with thrombosis
. Thromb Haemost
. 2002
;87
(5
):802
–7
.20.
Ruggeri
M
, Gisslinger
H
, Tosetto
A
, Rintelen
C
, Mannhalter
C
, Pabinger
I
, et al Factor V Leiden mutation carriership and venous thromboembolism in polycythemia vera and essential thrombocythemia
. Am J Hematol
. 2002
;71
(1
):1
–6
. .21.
Gisslinger
H
, Müllner
M
, Pabinger
I
, Heis-Vahidi-Fard
N
, Gisslinger
B
, Brichta
A
, et al Mutation of the prothrombin gene and thrombotic events in patients with polycythemia vera or essential thrombocythemia: a cohort study
. Haematologica
. 2005
;90
(3
):408
–10
.22.
Tevet
M
, Ionescu
R
, Dragan
C
, LUPU
AR
. Influence of the JAK2 V617F mutation and inherited thrombophilia on the thrombotic risk among patients with myeloproliferative disorders
. Maedica
. 2015
;10
(1
):27
.23.
De Stefano
V
, Vannucchi
AM
, Ruggeri
M
, Cervantes
F
, Alvarez-Larrán
A
, Iurlo
A
, et al Splanchnic vein thrombosis in myeloproliferative neoplasms: risk factors for recurrences in a cohort of 181 patients
. Blood Cancer J
. 2016
;6
(11
):e493
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2020
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