Department of Hematology, The Second Hospital of Tianjin Medical University, Tianjin 300211, China.
State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin, 300020, China; Tianjin Key Laboratory of Gene Therapy for Blood Diseases, Tianjin 300020, China; Center for Stem Cell Medicine, Chinese Academy of Medical Sciences, China; Department of Stem Cell & Regenerative Medicine, Peking Union Medical College, China.
Thromb Res. 2022 Apr;212:38-43. doi: 10.1016/j.thromres.2022.02.006. Epub 2022 Feb 12.
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.
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.
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 × 10/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.
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.
真性红细胞增多症（PV）后骨髓纤维化（Post-PV MF）是 PV 自然进展的晚期阶段。血栓形成是 PV 患者发病率和死亡率的主要原因；然而，Post-PV MF 中的血栓形成特征尚未确定。
分析了 163 例 Post-PV MF 患者的临床和实验室特征。采用 Kaplan-Meier 和多变量 Cox 分析来估计血栓形成的危险因素。
随访期间，84 例（51.5%）患者发生血栓形成，11 例（6.7%）进展为急性白血病，35 例（21.5%）死亡（20%因血栓形成）。Post-PV MF 患者的无血栓形成生存（TFS）明显低于性别和年龄匹配的 PV 患者（P<0.0001）。Post-PV MF 诊断后静脉血栓形成的发生率明显高于诊断前或诊断时；V617F%≥75%或绝对单核细胞计数（AMC）≥1.5×10/L 的患者发生静脉血栓形成的风险更高（P<0.05）。根据多变量 Cox 回归，可触及的脾肿大（危险比 [HR] 3.284 [95%置信区间（CI）1.373-7.855]；P=0.008）、年龄≥60 岁（HR 1.604 [95%CI 1.004-2.56]；P=0.048）、血栓形成史（HR 2.767 [95%CI 1.735-4.412]；P<0.001）是血栓形成的危险因素。在多变量模型中，Post-PV MF 极高危、高危、中危、低危组的中位 TFS 分别为 2、4、9 和 13 年。
Post-PV MF 患者血栓形成发生率较高。可触及的脾肿大、年龄≥60 岁、血栓形成史是血栓形成的独立危险因素。
