Guler Ekrem, Caliskan Umran, UcarAlbayrak Canan, Karacan Mehmet
Department of Pediatrics, Kahramanmaras Sutcu Imam University Faculty of Medicine, Kahramanmaras Turkey.
J Pediatr Hematol Oncol. 2007 Nov;29(11):783-5. doi: 10.1097/MPH.0b013e318159a570.
Thalassemias and sickle cell anemia (SCA) are common disease in Turkey. To determine the prevalence of beta-thalassemia and SCA traits in Konya urban area of Turkey, all couples applied for marriage procedures were screened. Screening tests included complete blood count and quantitation of hemoglobin for both partners. The subjects were considered to have the beta-thalassemia trait if they had a mean corpuscular volume of less than 80 fL and/or a mean corpuscular hemoglobin level of less than 27 pg and a hemoglobin A2 level of more than 3.2% or a hemoglobin F level of more than 2%. Subjects were considered to have an SCA trait if they were positive for sickle hemoglobin. During the study, premarital screening of hemoglobinopathies was evaluated retrospectively in 72,918 subjects; the thalassemia trait was detected in 1465 subjects (2%), and the SCA trait was detected in 37 subjects (0.05%). Of the carriers of the beta-thalassemia trait, 820 (56%) people had high hemoglobin A2, 513 (35%) people had high hemoglobin F, and 132 (9%) people had both high hemoglobin F and hemoglobin A2. Our results are very similar to Turkey's beta-thalassemia and SCA trait averages.
地中海贫血和镰状细胞贫血（SCA）在土耳其是常见疾病。为了确定土耳其科尼亚市区β地中海贫血和SCA特征的患病率，对所有申请结婚手续的夫妇进行了筛查。筛查测试包括对双方进行全血细胞计数和血红蛋白定量。如果受试者的平均红细胞体积小于80 fL和/或平均红细胞血红蛋白水平小于27 pg，且血红蛋白A2水平高于3.2%或血红蛋白F水平高于2%，则被认为具有β地中海贫血特征。如果受试者的镰状血红蛋白呈阳性，则被认为具有SCA特征。在研究期间，对72918名受试者进行了回顾性血红蛋白病婚前筛查；检测到1465名受试者（2%）具有地中海贫血特征，37名受试者（0.05%）具有SCA特征。在β地中海贫血特征携带者中，820人（56%）血红蛋白A2水平高，513人（35%）血红蛋白F水平高，132人（9%）血红蛋白F和血红蛋白A2水平均高。我们的结果与土耳其β地中海贫血和SCA特征的平均水平非常相似。
