大马士革国家地中海贫血症和遗传咨询中心转诊的贫血患者中的血红蛋白 C 紊乱症

Aya Ahmad, Karam Fattoum, Wael Imam, Mhd Yasser Mukhalalaty, Musab Murad, Faizah Ali Al Quobaili
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摘要

背景:血红蛋白病是地中海地区常见的遗传性血液疾病。由于血红蛋白 S 和血红蛋白 C 的发病率较高,因此主要的结构性血红蛋白变异体是血红蛋白 S 和血红蛋白 C。我们进行了这项回顾性研究,以调查和描述转诊至国家地中海贫血和遗传咨询中心的血红蛋白 C 患者以及大马士革血红蛋白 C 疾病的管理情况。材料和方法:研究对象包括 2000 年至 2022 年期间因检测出 C 型血红蛋白而转诊至大马士革国家地中海贫血和遗传咨询中心的患者。在对 C 型血红蛋白患者进行分类时,考虑了性别、年龄、地域、血红蛋白电泳图谱和输血情况。确定了连续五年的输血量以及与血红蛋白 S 和 C 值的线性回归。结果在 624 名 3 至 46 岁的患者中,有 30 人(男性 14 人,女性 16 人)患有血红蛋白 C 疾病(平均 ± SD:17.3 ± 9.7 岁)。只有 8 名患者(1 名男性和 7 名女性)接受了输血,其余患者(13 名男性和 9 名女性)未接受任何输血。只有一名患者的血红蛋白 C 含量为 100%;19 名患者的血红蛋白 C 含量为 HbSC,10 名患者的血红蛋白 C 含量为 HbAC。血红蛋白 S 与地理来源有明显的相关性(P 值=0)。结论在我们的研究中,同型血红蛋白 C 患者有轻度溶血性贫血,而血红蛋白 C 100%患者仅输血一次(17 岁)。血红蛋白 C 和血红蛋白 S 的遗传组合比单独的血红蛋白 S 严重程度要轻。血红蛋白 S 与地理来源之间有明显的关系(P 值=0)。
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Hemoglobin C Disorder in Anemic Patients Referred to the National Center for Thalassemia and Genetic Counseling in Damascus
Background: Hemoglobinopathies are common inherited blood disorders in our Mediterranean area. The main structural hemoglobin variants are hemoglobin S and hemoglobin C, due to their prevalence. We conducted this retrospective study to investigate and characterize hemoglobin C patients referred to the National Center for Thalassemia and Genetic Counseling and the management of hemoglobin C disease in Damascus. Materials and Methods: The study included patients referred to the National Center for Thalassemia and Genetic Counseling in Damascus between 2000 and 2022 for hemoglobin C detection. Gender, age, geographical origin, hemoglobin electrophoresis profile, and blood transfusion were considered for hemoglobin C patient classification. Blood transfusion in five consecutive years and linear regression with hemoglobin S and C values were determined. Results: 30 (14 males and 16 females) out of 624 patients between 3 and 46 years old (mean ± SD: 17.3 ± 9.7 years) showed hemoglobin C disease. Only eight patients (one male and seven females) received blood transfusions, and the remaining patients (13 males and 9 females) did not receive any transfusion. Only one patient with 100% hemoglobin C was detected; 19 showed HbSC, and 10 had HbAC. There was a significant correlation between hemoglobin S and geographical origin (P-value=0). Conclusion: A Homozygote hemoglobin C patient has mild hemolytic anemia, whereas the hemoglobin C 100% patient has only a one-time blood transfusion (he was 17 years old) in our study. The inherited combination of hemoglobin C and S is less severe than hemoglobin S alone. There is a significant relationship between hemoglobin S and geographical origin (p-value=0).
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