中间型β地中海贫血治疗现状及并发症单中心调查  

Investigation of treatment and complications in patients with beta-thalassaemia intermediate from single center in Guangxi

作　　者：黄琴[1,2] 张梅香[3] 聂伟业 李云双[1,2] 周亚丽[2] 陈永升[2] 张新华[2] 尹晓林[2] HUANG Qin ZHANG Meixiang NIE Weiye LI Yunshuang ZHOU Yali CHEN Yongsheng ZHANG Xinhua YIN Xiaolin(Graduate School of Guilin Medical College,Guilin, 541001, China Department of Hemotolo- gy,303rd Hospital of the PLA Department of Hematology, Peking University International Hospital)

机构地区：[1]桂林医学院研究生院,广西桂林541001 [2]中国人民解放军第303医院血液科 [3]北京大学国际医院血液科

出　　处：《临床血液学杂志》2016年第5期748-751,共4页Journal of Clinical Hematology

基　　金：广西科技攻关项目(No:1355005-2-1);2015年广西自然基金(No:2015GXNSFAA13916)

摘　　要：目的：了解本中心中间型β地中海贫血（β地贫）患者的治疗和并发症现状,探讨如何提高中间型β地贫患者的生存质量。方法：回顾性总结解放军第303医院2011-2014年收治的92例中间型β地贫患者的临床资料,调查患者的临床特征、治疗现状、并发症现状,分析其影响因素。结果：92例患者中,男51例（55.4%）,女41例（44.6%）,平均年龄（24.9±9.2）岁,起病年龄（4.0±5.8）岁（1-30岁）。91例患者接受过输血治疗（其中15例为规律输血）,接受输血者平均血红蛋白为（58.1±20.1）g/L。72例患者有铁超载,但仅22例有接受去铁治疗史。平均血清铁蛋白为（2 172.0±3 728.1）（344-12 482）ng/ml,40例（43.5%）为重度铁超载者（血清铁蛋白〉2 500ng/ml）。骨质疏松、肺动脉高压、肝功能异常、胆囊结石、髓外造血的发生比例分别为54.1%、55.7%、43.3%、44.1%和9.7%。结论：广西地区本中心中间型β地贫患者的治疗很不充分,并发症发生率高。需加强对中间型β地贫患者的输血和去铁治疗,以提高患者生存质量。Objective：To investigate the treatment status and complications of beta-thalassaemia intermediate（TI）and explore how to improve the prognosis of patients with TI.Method：It was a cross-section study.From2011 to 2014,92 patients with TI treated in the 303 rd Hospital of PLA were included.Clinical features,treatment status and complications were investigated.Risk factors of complications were analyzed.Result：Among 92 patients,there were 51 males（55.4%）and 41 females（44.6%）,with mean age of（24.9±9.2）years.The mean age of onset was（4.0±5.8）years（ranges 1 to 30）.All excluding one patient received blood transfusion,among them 15 patients were transfused regularly.The average hemoglobin in patients with blood transfusion was（58.1±20.1）g/L.Iron overload was founded in 72 patients,but iron chelating therapy was performed in only 22 cases.The mean level of serum ferritin（SF）was（2 172.0±3 728.1）ng/ml（ranges 344 to 12 482ng/ml）,with 40 cases（43.5%）diagnosed severe iron overload（SF2 500ng/ml）.The proportion of osteoporosis,pulmonary hypertension,abnormal liver function,gallstones and extramedullary hematopoiesis were 54.1%,55.7%,43.3%,44.1% and 9.7%,respectively.Conclusion：The treatment of patients with TI in Guangxi is limited,and the incidence of complications is high.Cognition and treatment should be strengthened to improve the quality of survival in patients with TI.

关 键 词：中间型β地中海贫血 输血 铁超载 并发症 

分 类 号：R556[医药卫生—血液循环系统疾病]