Highlights
- •Patients with post-PV MF demonstrated a higher incidence of thrombosis, especially venous thrombosis.
- •JAK2V617F allele burden ≥75% associated with venous thrombosis in post-PV MF.
- •≥1 DTA gene mutation in post-PV MF may increase the risk for thrombosis, especially the ASXL1 mutation.
- •Palpable splenomegaly was a risk factor for thrombosis in post-PV MF.
- •According to risk factor for thrombosis, a prognostic model was developed in post-PV MF.
Abstract
Introduction
Post-polycythemia vera (PV) myelofibrosis (Post-PV MF) is an advanced phase of natural
progression of PV. Thrombosis is a major cause of morbidity and mortality in PV; however,
the characteristics of thrombosis in post-PV MF have not been characterised.
Methods
The clinical and laboratory characteristics of 163 patients with post-PV MF were analysed.
Kaplan–Meier and multivariate Cox analyses were used to estimate risk factors for
thrombosis.
Results
During follow-up, 84 (51.5%) patients developed thrombosis, 11 (6.7%) progressed to
acute leukemia, 35 (21.5%) died (20% due to thrombosis). Thrombosis-free survival
(TFS) in post-PV MF was lower than that of sex- and age-matched patients with PV (P < 0.0001). The incidence of venous thrombosis was significantly higher after diagnosis
of post-PV MF than before or at diagnosis; Those with V617F% ≥ 75% or absolute monocyte count (AMC) ≥1.5 × 109/L demonstrated a higher risk for venous thrombosis (P < 0.05). According to multivariate Cox regression, palpable splenomegaly (hazard
ratio [HR] 3.284 [95% confidence interval (CI) 1.373–7.855]; P = 0.008), age ≥ 60 years (HR 1.604 [95%CI 1.004–2.56]; P = 0.048), history of thrombosis (HR 2.767 [95%CI 1.735–4.412]; P < 0.001) were risk factors for thrombosis. In multivariable models, median TFS in
post-PV MF in extremely high -, high -, intermediate -, low-risk groups were 2, 4,
9 and 13 years, respectively.
Conclusion
Patients with post-PV MF demonstrated a higher incidence of thrombosis. Palpable splenomegaly,
age ≥ 60 years, history of thrombosis were independent risk factors for thrombosis.
Keywords
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Article info
Publication history
Published online: February 12, 2022
Accepted:
February 7,
2022
Received in revised form:
January 27,
2022
Received:
August 8,
2021
Identification
Copyright
© 2022 Elsevier Ltd. All rights reserved.