Pathology Division, Department of Medical Research, Yangon, Myanmar.
Southeast Asian J Trop Med Public Health. 1992 Jun;23(2):264-8.
The present study was conducted in the Outpatient Department (OPD) of Yangon Children's Hospital (YCH) during June to November 1990 to determine the hematological data of 133 Myanmar patients with thalassemia trait who were the parents of patients with known beta-thalassemia major or hemoglobin E (Hb E)/beta-thalassemia. The mean values of hemoglobin (Hb) concentration, packed cell volume (PCV), mean cell hemoglobin (MCH) and mean cell volume (MCV) were significantly lower than normal controls but the mean cell hemoglobin concentration (MCHC) was the same as controls. Increased osmotic resistance tested in 0.36% buffered saline was detected in 81-97% of cases depending on the cut-off point. High levels of Hb A2 (> 3.5%) were found in 93% of cases whereas Hb F was increased (> 0.8%) only in 23% of cases. Although the mean red cell count (RBC) was significantly higher than normal, only 79% of thalassemia traits were detected if the RBC count of > 5.0 x 10(12)/1 was taken as the discrimination limit. Other discrimination functions such as MCH/RBC, MCV/RBC, (MCV)2 x MCH x 0.01 and MCV-(RBC/10(12)/1)-(5 x Hb) - 3.4 or - 8.4 were tested for their utility in diagnosing thalassemia traits. All of them were found not to be superior to each of the simple tests (MCV, MCH, Hb A2 or osmotic fragility) in diagnosing thalassemia traits. The one tube osmotic fragility test is a the suitable test to be used in future thalassemia screening programs in Myanmar.
本研究于1990年6月至11月在仰光儿童医院门诊部进行，旨在确定133名缅甸轻型地中海贫血患者的血液学数据，这些患者是已知重型β地中海贫血或血红蛋白E（Hb E）/β地中海贫血患者的父母。血红蛋白（Hb）浓度、红细胞压积（PCV）、平均红细胞血红蛋白含量（MCH）和平均红细胞体积（MCV）的平均值显著低于正常对照组，但平均红细胞血红蛋白浓度（MCHC）与对照组相同。根据临界值，在0.36%缓冲盐水中进行的渗透抵抗力增加检测，81%至97%的病例中检测到。93%的病例中发现高水平的Hb A2（>3.5%），而只有23%的病例中Hb F升高（>0.8%）。虽然平均红细胞计数（RBC）显著高于正常水平，但如果将RBC计数>5.0×10¹²/L作为判别界限，仅检测到79%的轻型地中海贫血。还测试了其他判别函数，如MCH/RBC、MCV/RBC、（MCV）²×MCH×0.01以及MCV - （RBC/10¹²/L） - （5×Hb） - 3.4或 - 8.4在诊断轻型地中海贫血中的效用。结果发现，在诊断轻型地中海贫血方面，它们都不比任何一项简单检测（MCV、MCH、Hb A2或渗透脆性）更优越。单管渗透脆性试验是缅甸未来地中海贫血筛查项目中适用的检测方法。
