原发性骨髓纤维化患者右心功能减退临床指标变化分析  

Analysis of clinical index changes of right heart hypofunction in patients with primary myelofibrosis

作　　者：柴星星[1] 杜晓鹏 何耀[1] 庄万传[1] 刘晓庆 程朗 刘正媛 何广胜[2] 李建勇[2] Chai Xingxing;Du Xiaopeng;He Yao;Zhuang Wanchuan;Liu Xiaoqing;Cheng Lang;Liu Zhengyuan;He Guangsheng;Li Jianyong(Department of Hematology,the Second People's Hospital of Lianyungang in Jiangsu Province,Lianyungang 222000,China;Department of Hematology,the First Affiliated Hospital of Nanjing Medical University,Jiangsu Province Hospital,Nanjing 210029,China)

机构地区：[1]江苏省连云港市第二人民医院血液科,222000 [2]南京医科大学第一附属医院江苏省人民医院血液科,南京210029

出　　处：《白血病．淋巴瘤》2021年第5期272-276,共5页Journal of Leukemia & Lymphoma

基　　金：国家中医药管理局行业专项(201407001⁃4);卫生部科研基金(201202017);江苏省医学重点项目(BL2014086);江苏省普通高校优势学科(JX10231801)。

摘　　要：目的探讨原发性骨髓纤维化(PMF)患者右心功能减退相关指标变化。方法回顾性分析2015年1月至2019年8月江苏省连云港市第二人民医院和江苏省人民医院55例PMF患者临床资料。比较纤维化前/早期和明显纤维化期患者右心功能相关超声心动图指标和生化指标差异,采用单因素线性回归法分析肺动脉压与各生化指标的关系。结果纤维化前/早期组患者血红蛋白水平[119 g/L(47~224 g/L)比78 g/L(33~182 g/L)]及血小板计数[233×10^(12)/L(5×1012/L~984×10^(12)/L)比117×10^(12)/L(7×1012/L~731×10^(12)/L)]高于明显纤维化期组,差异均有统计学意义(均P<0.05)。22例有完整心脏超声结果的患者中,90.9%(20/22)患者肺动脉压增高,72.7%(16/22)患者左心房直径增加,90.9%(20/22)患者右心室舒张期内径增加,无射血分数异常者。纤维化前/早期组患者肺动脉压[48 mmHg(46~90 mmHg)比33 mmHg(20~50 mmHg)(1 mmHg=0.133 kPa)]、左心室舒张期内径[46 mm(36~50 mm)比47 mm(43~53 mm)]、短缩率[38.1%(36.0%~38.9%)比35.4%(32.7%~37.8%)]与明显纤维化期组比较,差异均有统计学意义(均P<0.05)。患者肺动脉压与年龄(r=0.590)及血清铁蛋白(SF)(r=0.608)、乳酸脱氢酶(LDH)(r=0.711)、可溶性生长刺激表达基因2(ST⁃2)(r=0.580)水平均呈正相关(均P<0.05),与血小板计数呈负相关(r=-0.596,P=0.003)。结论PMF患者易出现右心功能减退。PMF患者年龄越高、血小板计数越低或SF、LDH、ST⁃2水平升高时,肺动脉压越高。Objective To investigate the changes of related indicators of right heart hypofunction in patients with primary myelofibrosis(PMF).Methods The clinical data of 55 PMF patients in the Second People's Hospital of Lianyungang in Jiangsu Province and Jiangsu Province Hospital from January 2015 to August 2019 were retrospectively analyzed.The differences in right heart function⁃related echocardiographic indexes and biochemical indexes between pre⁃fibrosis/early stage fibrosis patients and obvious stage fibrosis patients were compared.Single factor linear regression method was used to analyze the correlations of pulmonary artery pressure with biochemical indexes.Results The hemoglobin level[119 g/L(47-224 g/L)vs.78 g/L(33-182 g/L)]and platelet count[233×10^(12)/L(5×10^(12)/L-984×10^(12)/L)vs.117×10^(12)/L(7×10^(12)/L-731×10^(12)/L)]of patients in the pre⁃fibrosis/early stage fibrosis group were higher than those in the obvious stage fibrosis group,and the differences were statistically significant(both P<0.05).Among 22 patients with complete results of cardiac ultrasound,90.9%(20/22)patients had increased pulmonary artery pressure,72.7%(16/22)patients had increased left atrial diameter,and 90.9%(20/22)patients had increased right ventricular diastolic diameter.There were no patients with abnormal ejection fraction.The pulmonary artery pressure[48 mmHg(46-90 mmHg)vs.33 mmHg(20-50 mmHg)(1 mmHg=0.133 kPa)],left ventricular diastolic diameter[46 mm(36-50 mm)vs.47 mm(43-53 mm)]and fractional shortening rate[38.1%(36.0%-38.9%)vs.35.4%(32.7%-37.8%)]of patients in the pre⁃fibrosis/early stage fibrosis group were higher than those in the obvious stage fibrosis group,and the differences were statistically significant(all P<0.05).The pulmonary artery pressure of patients had positive correlations with age(r=0.590),serum ferritin(SF)(r=0.608),lactate dehydrogenase(LDH)(r=0.711)and soluble growth⁃stimulating expression gene 2(ST⁃2)(r=0.580)(all P<0.05),and had negative correlation with platelet count(r=-0.596,P=0.

关 键 词：原发性骨髓纤维化 心室功能 右 心房功能 右 高血压 肺性 乳酸脱氢酶类 铁蛋白质类 

分 类 号：R733.3[医药卫生—肿瘤]