1例合并G6PD缺乏的HbH患者急性溶血期血液学参数分析

通过1例合并G6PD缺乏的HbH患者急性溶血期的实验室检查结果,分析其血液学参数在两种疾病同时存在并急性溶血发作时的相互影响以及对诊断治疗的意义。通过对此患者的诊治,得出如下结论:(1)溶血性患者MCV正常或是增大均不能排除地中海贫血的可能,需进行地中海贫血相关检查加以确证;(2)在急性溶血期时G6PD检测值正常的患者不能排除G6PD缺乏症,需待急性溶血纠正1个月后再复查G6PD活性才能得出真实结果。临床诊断及治疗时不可盲信一时的实验室结果,需多方面综合考虑,谨慎处理防止溶血加重。由于此两种疾病均为遗传性疾病,因此预防及治疗此类疾病的最有效方法是:积极婚检、产检以避免有重症缺陷的患儿出生。

Clinical Hematology Analysis of A Patient with HbH Complicated with G6PD Deficiency in Acute Hemolysis

Through the laboratory examination results of a case with HbH complicated with G6PDdeficiency in acute hemolysis,to analysis the interaction of hematology parameters when the twodiseases exist at the same time and complicated with acute hemolysis,and investigates the meaningto diagnosis and therapy. Through this case,obtains the conclusions as below：（1）In hemolyticpatient,with MCV normal or higher,it can not exclude the possibility of thalassemia,and need tohave further examination to confirm;（2）In acute hemolysis,with G6PD normal,it can not excludethe possibility of G6PD deficiency,need to reexam G6PD 1 month after acute hemolysis becomenormal,to obtain the real result. The clinical diagnosis and therapy can not believe the temporaryresults of laboratory fanatically,and should consider comprehensively,so cautiously treats thehemolysis and avoids becoming worse. Because that the both two are genetic disease,the mosteffective way to prevent and treat the disease are antemarital physical examination and prenatalexamination so as to avoid the birth of infant with the defect.