Thrombohemorrhagic events, disease progression, and survival in polycythemia vera and essential thrombocythemia: a retrospective survey in Miyazaki prefecture, Japan
Thrombohemorrhagic events, disease progression, and survival in polycythemia vera and essential thrombocythemia: a retrospective survey in Miyazaki prefecture, Japan
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真性红细胞增多症和原发性血小板增多症的血栓出血事件、疾病进展和生存：日本宫崎县的回顾性调查
DOI：
10.1007/s12185-018-2428-0
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发表时间：
2018
期刊：
影响因子：
2.1
通讯作者：
Shimoda K.
中科院分区：
文献类型：
--
作者：
Kamiunten A;Shide K;Kameda T;Sekine M;Kubuki Y;Ito M;Toyama T;Kawano N;Marutsuka K;Maeda K;Takeuchi M;Kawano H;Sato S;Ishizaki J;Akizuki K;Tahira Y;Shimoda H;Hidaka T;Yamashita K;Matsuoka H;Shimoda K.
真性红细胞增多症（PV）和原发性血小板增多症（ET）与危及生命的血栓出血事件有关，非血液系统恶性肿瘤的疾病进展和发展也会降低长期生存率。我们回顾性调查了62例PV和117例ET患者的血栓出血事件和总生存率（OS）。PV和ET患者血栓出血性事件的累积发生率分别为11.3和10.3%，发病率分别为2.42和1.85 / 100人年。PV和ET患者疾病进展和非血液学恶性肿瘤发展的总发病率分别为1.73和1.69 / 100人年。我们的日本PV/ET患者的血栓出血性事件发生率低于大多数西方研究报告的发生率，但与最大的ET患者前瞻性观察性研究的发生率相当。在日本和西方PV/ET患者中，疾病进展和非血液系统恶性肿瘤的综合发病率相似。在ET患者中，基于高龄或血栓形成史的传统风险分层模型可用于预测血栓形成风险，基于上述2个危险因素加上白细胞计数升高的传统模型和ET血栓形成国际预后评分均可预测预后不良。
Polycythemia vera (PV) and essential thrombocythemia (ET) are associated with life-threatening thrombohemorrhagic events, and disease progression and development of non-hematological malignancies also reduce long-term survival. We retrospectively surveyed thrombohemorrhagic events and overall survival (OS) in 62 PV and 117 ET patients. The cumulative incidences of thrombohemorrhagic events in PV and ET patients were 11.3 and 10.3%, and the incidence rates were 2.42 and 1.85 per 100 person-years. The combined incidence rates of disease progression and development of non-hematological malignancies in PV and ET patients were 1.73 and 1.69 per 100 person-years. The incidence rates of thrombohemorrhagic events in our Japanese PV/ET patients were lower than those reported by most Western studies, but were comparable to those in the largest prospective observational study in ET patients. The combined incidence rates of disease progression and development of non-hematological malignancies were similar between Japanese and Western PV/ET patients. In ET patients, the conventional risk stratification model based on the presence of advanced age or history of thrombosis was useful to predict thrombosis risk, and both the conventional model and the International Prognostic Score of thrombosis in ET based on the above 2 risk factors plus increased leukocyte count could predict poor survival.