新疆维吾尔自治区不同民族真性红细胞增多症临床分析

Clinical analysis of polycythemia vera among different nationalities in Xinjiang Uygur Autonomous Region

目的观察新疆维吾尔自治区维吾尔族和汉族真性红细胞增多症（PV）患者的临床表现和自然病程。方法分析37例（汉族20例、维吾尔族13例、其他民族4例）PV患者的临床表现、体征、实验室检查和预后转归。结果37例患者中男22例，女15例。年龄31～85岁，平均年龄（59．41±11．31）岁。平均血红蛋白水平（191．11±21．41）g／L。红细胞增多伴有白细胞增多和血小板增多者分别为23例（62．2％）和19例（51．4％）。脾大27例（73．0％），肝大9例（24．3％）。血清乳酸脱氢酶（LDH）升高28例（75．7％）。发生血栓事件22例（59．5％），发展为骨髓纤维化（MF）2例，未出现第二肿瘤者，死于血栓事件2例。年龄〉60岁、自细胞增多、血小板增多者血栓事件发生危险显著增加。维吾尔族和汉族PV患者之间临床表现和血栓事件发生率差异无统计学意义（P〉0．05）。结论年龄〉60岁、白细胞增多、血小板增多可能是PV患者发生血栓事件的高危危险因素，新疆维吾尔自治区维吾尔族和汉族PV患者的临床表现和自然病程差异无统计学意义（19〉0．05）。

Objective To investigate and compare the clinical feature and natural course of polycythemia vera （PV） between Uygur and HaM nationalities in Xinjiang Uygur Autonomous Region. Methods Clinical symptoms, signs, laboratory tests and prognosis of 37（20 HaM, 13 Uygur, 4 other nationalities） patients with PV were analyzed. Results There were 22 males and 15 female. The mean age of all patients was 59.41±11.31（31-85） years old. The mean hemoglobulin level was （191.11±21.41） g/L. Erythremia combined with leukocytosis or thrombocytosis were observed in 23 （62.2 %） or 19 （51.4 %） patients respectively. Splenomegaly was found in 27 （73.0 %） patients and hepatomegaly in 9（24.3 %）. Serum LDH was elevated in 28 （75.7 %） patients. Twenty two patients （59.5 %） had vascular thrombotic events. Two patients developed MF. No secondary neoplasm was identified. Two patients died of thrombosis. Over 60 years old, leukocytosis and thrombocytosis were the high risk factors associated with thrombosis. No significant differences of clinical manifestations and thrombotic events have been found between Uygur and HaM patients in Xinjiang. Conclusion Over 60 years old, leukocytosis and thrombocytosis were probably the high risk factors of thrombosis in PV patients. There was no significant difference in clinical feature and natural course of PV between Uygur and HaM nationalities in Xinjiang Uygur Autonomous Region.