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真性红细胞增多症患者发生血栓形成、骨髓纤维化及演变为恶性肿瘤的长期风险因素:来自中国的单中心经验

Risk factors of long-term incidences of thrombosis, myelofibrosis and evolution into malignance in polycythemia vera: a single center experience from China.

作者信息

Bai Jie, Xue Yangping, Ye Lei, Yao Jianfeng, Zhou Chunlin, Shao Zonghong, Qian Linsheng, Yang Renchi, Li Haiyan, Zhang Hongyun, Zheng Yizhou

机构信息

Polycythemia Vera Studying Program, Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Science and Peking Union Medical College, 288 Nanjing Road, 300020, Tianjin, People's Republic of China.

出版信息

Int J Hematol. 2008 Dec;88(5):530-535. doi: 10.1007/s12185-008-0188-y. Epub 2008 Nov 11.

DOI:10.1007/s12185-008-0188-y
PMID:19002391
Abstract

To find out risk factors of incidences of long-term complications of thrombosis, myelofibrosis with myeloid metaplasia (MMM) and evolution into malignance in Chinese PV patients, we evaluated 320 PV patients referred to our center from April 1984 to June 2005 by Kaplan-Meier estimation and Cox proportional hazards models. A total of 250 events of thrombosis were observed in 138 (43.13%) patients. Advanced age, prior thrombosis and hemoglobin out of control were statistically significant risk factors of incidences of thrombotic events. A total of 43 patients progressed into MMM at a median time of 84 (7-240) months, higher white blood cell (WBC) count, splenomegaly, receiving alkylating agent and hydroxycarbamide were associated with the progression into MMM. During the follow-up time, 11 and 2 patients died of fatal complications of thrombosis and acute myeloid leukaemia (AML), respectively. These results suggest that advanced age, prior thrombosis and hemoglobin out of control contributed to relatively high incidence of thromboembolism; higher WBC count, splenomegaly, receiving alkylating agent and hydroxycarbamide were risk factors of evolution to MMM. The main poor factors that influenced the survival of Chinese PV patients were incidences of thromboembolism and evolution into AML.

摘要

为了找出中国真性红细胞增多症(PV)患者发生血栓形成、骨髓纤维化伴髓外化生(MMM)等长期并发症以及病情恶化为恶性肿瘤的风险因素,我们采用Kaplan-Meier估计法和Cox比例风险模型,对1984年4月至2005年6月转诊至我院的320例PV患者进行了评估。共观察到138例(43.13%)患者发生250次血栓事件。高龄、既往有血栓形成史以及血红蛋白失控是血栓事件发生率的统计学显著风险因素。共有43例患者进展为MMM,中位时间为84(7 - 240)个月,白细胞(WBC)计数较高、脾肿大、接受烷化剂和羟基脲与进展为MMM相关。在随访期间,分别有11例和2例患者死于血栓形成的致命并发症和急性髓细胞白血病(AML)。这些结果表明,高龄、既往有血栓形成史以及血红蛋白失控导致血栓栓塞发生率相对较高;白细胞计数较高、脾肿大、接受烷化剂和羟基脲是进展为MMM的风险因素。影响中国PV患者生存的主要不良因素是血栓栓塞的发生率和恶化为AML。

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