22ql1微缺失综合征的血小板特征及其临床应用初探

The platelet characteristics of 22qll microdeletion and its clinical application

目的探讨22q11先心病患者的血小板特征，及其在心脏手术中可能的临床应用价值。方法选择已行心脏手术的80例患儿作为研究对象，所有患儿均经原位荧光杂交（FISH）检测明确为22q11微缺失综合征40例，阴性者40例。收集医院信息系统中患儿术前检查中血小板相关数据，进行统计学分析。结果22qll微缺失综合征平均血小板体积明显大于对照组[（11．20±1．94）fLvs（8．95±1．58）fL，P〈0．01]。平均血小板体积的接收者操作特征曲线下面积为0．82，具有预测意义。平均血小板体积（MPV）=10fL的敏感度为70．0％，特异度为80．0％。结论22q11先心病患者平均血小板体积显著大于非22qll先心病患者，并可从血常规中获得数据，可以作为22q11的经济、快捷的初筛手段。MPV=10fL可以作为心脏手术给辐照血的参考指标。

[ Abstract] Objective 22ql 1 microdeletion is featured with hemotological dysfunction, among oth- ers： hypoealcemia, thrombocytopenia and megathrombocyte. This study was trying to work out the platelet characteristics of 22ql 1 microdeletion patients and its potential clinical application： Methods A total of 80 cases who had undergone open -heart surgery were selected and confirmed with fluorescence in situ hybrid- ization （FISH） to have 22qll microdeletion among 40 cases while the others were negative. The relevant data in full blood routine of all the 80 cases in hospital information system （HIS） then were collected and analyzed with suitable statistical methods. Results The mean platelet volume in microdeletion group was statistically higher than that in the control group [ （ 11.20 ＋ 1.94）fL vs （8. 95 ＋ 1.58） fL, P 〈0.01 ]. The area under the receiver operating characteristic （ROC） curve was 0. 82, meaning significant predictive val- ues. The corresponding sensitivity and specificity for mean platelet volume （MPV） = 10 fL were 70. 0 % and 80.0 %, respectively. Conclusions MPV in congenital heart defect patients with 22qll is significant- ly higher than those without 22ql 1. It is an effective method for preliminary screening 22ql 1. Being ob- tained from full blood routine data, it is economic and quick. MPV = 10 fL can be used as a cutoff for guid- ance for irradiated blood transfusion postoperatively.