Predicting factors of survival rates among alpha- and beta-thalassemia patients: a retrospective 10-year data analysis

Kunapa Iam-arunthai, T. Suwanban, Pravinwan Thungthong, Supat Chamnanchanunt, Suthat Fucharoen
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Abstract

Thalassemia is a genetic hemoglobinopathy in which a defective globin chain can cause transfusion-dependent anemia and other complications. As genotype interactions lead to variations in the clinical course among patients with thalassemia, clinical factors may help predict survival in the types of thalassemia complicated by gene interactions.This study aimed to determine the clinical factors associated with survival in patients with thalassemia. We retrospectively reviewed the medical records of patients with thalassemia older than 15 years between 2002 and 2020 that were available at the Rajavithi Hospital. Data on the clinical parameters, laboratory tests, treatments, and survival status were collected and analyzed.Of the 478 thalassemia patients included, 68.8% were women, and the mean age was 41 ± 17 years. The most common type of thalassemia was β-thalassemia (53.3%). Male sex, low body mass index, the thalassemia type, comorbidities, low hemoglobin level, high ferritin level, and regular blood transfusion were significantly associated with short-term survival. However, only the thalassemia type (β-thalassemia, p = 0.048) and the co-inheritance of the α- and β-thalassemia genotypes (p = 0.03) were independently associated with death. The overall survival rates among patients with α-thalassemia, β-thalassemia, and co-inheritance of the α- and β-thalassemia genotypes were 98.1%, 90.6%, and 75.0%, respectively. The death rate was 6.3%, and the most common cause of death was infection.The thalassemia genotype was a predictive factor of survival, and co-inheritance of the α- and β-thalassemia genotypes results in a shorter-term survival compared with other types, especially transfusion-dependent thalassemia. These results can be applied in clinical settings to predict and possibly extend the life expectancy of patients with thalassemia.
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甲型和乙型地中海贫血患者生存率的预测因素:10 年回顾性数据分析
地中海贫血是一种遗传性血红蛋白病,其球蛋白链缺陷可导致输血依赖性贫血和其他并发症。由于基因型相互作用会导致地中海贫血患者的临床病程发生变化,因此临床因素可能有助于预测因基因相互作用而复杂化的地中海贫血类型的存活率。我们回顾性地查看了拉贾维提医院在 2002 年至 2020 年期间 15 岁以上地中海贫血患者的病历。在纳入的 478 名地中海贫血患者中,68.8% 为女性,平均年龄为 41 ± 17 岁。最常见的地中海贫血类型是β地中海贫血(53.3%)。男性、低体重指数、地中海贫血类型、合并症、低血红蛋白水平、高铁蛋白水平和定期输血与短期存活率显著相关。然而,只有地中海贫血类型(β-地中海贫血,p = 0.048)以及α-和β-地中海贫血基因型的共同遗传(p = 0.03)与死亡有独立关联。α地中海贫血、β地中海贫血以及α和β地中海贫血基因型共同遗传患者的总生存率分别为98.1%、90.6%和75.0%。地中海贫血基因型是预测存活率的一个因素,与其他类型的地中海贫血,尤其是输血依赖型地中海贫血相比,α-和β-地中海贫血基因型的共同遗传导致存活期缩短。这些结果可应用于临床,以预测并可能延长地中海贫血患者的预期寿命。
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