Torcharus K, Sriphaisal T, Krutvecho T, Suwanasophon C, Intarapakawong J
Department of Pediatrics, Pramongkutklao College of Medicine, Bangkok, Thailand.
Southeast Asian J Trop Med Public Health. 1995;26 Suppl 1:275-7.
Thalassemia is a relatively common hemolytic anemia in Southeast Asia. Alpha and beta thalassemia, hemoglobin (Hb) E, and Hb Constant Spring (CS) are prevalent in Thailand. Different gene combinations lead to over 60 thalassemic syndromes. One hundred and forty-nine thalassemia families were retrospectively studied. They were 4 homozygous beta-thalassemia (beta-thal/ beta-thal), 79 beta-thal/Hb E, 22 Hb H disease, 32 Hb with Hb CS, and 6 AE Bart's disease. The first clinical manifestation and hematologic data including hemoglobin electrophoresis were analysed. Most homozygous beta-thalassemia and beta-thal/Hb E presented with anemia (100% vs 81%), hepatomegaly (40% vs 21%), and splenomegaly (20% vs 27%). In Hb H disease and Hb H with Hb CS, the clinical findings were anemia (74% vs 79%), hepatomegaly (9% vs 8%), splenomegaly (9% vs 13%), jaundice (24% vs 13%), and fever (18% vs 25%). The 317 hematologic data and hemoglobin types of the patients, their parents and relative were also analyzed. These findings can be used as reference values for childhood thalassemia and heterozygous states.
地中海贫血是东南亚一种相对常见的溶血性贫血。α和β地中海贫血、血红蛋白（Hb）E以及Hb Constant Spring（CS）在泰国较为普遍。不同的基因组合导致了60多种地中海贫血综合征。对149个地中海贫血家庭进行了回顾性研究。其中有4个纯合子β地中海贫血（β-地贫/β-地贫）家庭、79个β-地贫/Hb E家庭、22个Hb H病家庭、32个携带Hb CS的Hb家庭以及6个AE Bart病家庭。分析了首发临床表现以及包括血红蛋白电泳在内的血液学数据。大多数纯合子β地中海贫血和β-地贫/Hb E患者表现为贫血（分别为100%和81%）、肝肿大（分别为40%和21%）以及脾肿大（分别为20%和27%）。在Hb H病和携带Hb CS的Hb H患者中，临床表现为贫血（分别为74%和79%）、肝肿大（分别为9%和8%）、脾肿大（分别为9%和13%）、黄疸（分别为24%和13%）以及发热（分别为18%和25%）。还分析了患者及其父母和亲属的317份血液学数据和血红蛋白类型。这些研究结果可作为儿童地中海贫血及杂合状态的参考值。
