CAJ | 학술논문

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。
통과1례합병G6PD결핍적HbH환자급성용혈기적실험실검사결과，분석기혈액학삼수재량충질병동시존재병급성용혈발작시적상호영향이급대진단치료적의의。통과대차환자적진치，득출여하결론：（1）용혈성환자MCV정상혹시증대균불능배제지중해빈혈적가능，수진행지중해빈혈상관검사가이학증；（2）재급성용혈기시G6PD검측치정상적환자불능배제G6PD결핍증，수대급성용혈규정1개월후재복사G6PD활성재능득출진실결과。림상진단급치료시불가맹신일시적실험실결과，수다방면종합고필，근신처리방지용혈가중。유우차량충질병균위유전성질병，인차예방급치료차류질병적최유효방법시：적겁혼검、산검이피면유중증결함적환인출생。
Through the laboratory examination results of a case with HbH complicated with G6PD deficiency in acute hemolysis,to analysis the interaction of hematology parameters when the two diseases exist at the same time and complicated with acute hemolysis,and investigates the meaning to diagnosis and therapy. Through this case,obtains the conclusions as below:(1) In hemolytic patient,with MCV normal or higher,it can not exclude the possibility of thalassemia,and need to have further examination to confirm;(2)In acute hemolysis,with G6PD normal,it can not exclude the possibility of G6PD deficiency,need to reexam G6PD 1 month after acute hemolysis become normal,to obtain the real result. The clinical diagnosis and therapy can not believe the temporary results of laboratory fanatically , and should consider comprehensively , so cautiously treats the hemolysis and avoids becoming worse. Because that the both two are genetic disease , the most effective way to prevent and treat the disease are antemarital physical examination and prenatal examination so as to avoid the birth of infant with the defect.