Aldwaik Reem, Abu Mohor Tamara, Idyabi Israa, Warasna Salam, Abdeen Shatha, Karmi Bashar, Abu Seir Rania
Department of Medical Laboratory Sciences, Al-Quds University, Jerusalem, Palestine.
Thalassemia Patients' Friends Society, Ramallah, Palestine.
Front Med (Lausanne). 2021 Dec 20;8:788758. doi: 10.3389/fmed.2021.788758. eCollection 2021.
Management of β-thalassemia in developing countries is demanding in the absence of available therapies rather than recurrent transfusions. This study describes the characteristics and evaluates the hematological, biochemical, and hormonal findings of patients with β-thalassemia in the West Bank. We conducted a retrospective cohort study between January 2017 and December 2018. Data were collected through medical files of the patients with β-thalassemia from eight primary healthcare clinics, nine emergency departments, and 11 governmental hospitals across the West Bank. Results of the hematological, biochemical, and hormonal evaluations, in addition to demographic data and the use of iron chelation were included in the study and analyzed. A total of 309 patients with β-thalassemia were included with a male-to-female ratio of 1:1 and an average age of 23.4 ± 10.4 years. The anemic presentation was reported in 78.6% of the patients as indicated by hemoglobin level (mean ± SD = 8.4 ± 1.4 g/dl), and 73.1% had iron overload with serum ferritin (SF) levels ≥ 1,000 μg/L (mean ± SD = 317.8 ± 3,378.8 μg/L). Evaluation of the liver function tests showed that alanine transaminase (ALT) and aspartate transaminase (AST) levels were high among 38.1 and 61.2% of the patients, respectively. ALT and AST showed significant positive correlations with SF levels, while the kidney tests did not. As for iron chelation medications, patients receiving deferoxamine (26.5%) showed significantly higher SF levels compared with patients receiving deferasirox (73.5%). This study highlights the importance of establishing patient-tailored comprehensive assessment and follow-up protocols for the management of β-thalassemia with an emphasis on blood transfusion and iron chelation practices.
在缺乏有效治疗方法而非反复输血的情况下，发展中国家β地中海贫血的管理颇具挑战性。本研究描述了约旦河西岸β地中海贫血患者的特征，并评估了他们的血液学、生化和激素检查结果。我们在2017年1月至2018年12月期间进行了一项回顾性队列研究。数据通过约旦河西岸八家初级保健诊所、九个急诊科和11家政府医院的β地中海贫血患者的医疗档案收集。研究纳入并分析了血液学、生化和激素评估结果，以及人口统计学数据和铁螯合剂的使用情况。总共纳入了309例β地中海贫血患者，男女比例为1:1，平均年龄为23.4±10.4岁。根据血红蛋白水平（平均值±标准差=8.4±1.4g/dl），78.6%的患者有贫血表现，73.1%的患者血清铁蛋白（SF）水平≥1000μg/L，存在铁过载（平均值±标准差=317.8±3378.8μg/L）。肝功能检查评估显示，分别有38.1%和61.2%的患者丙氨酸转氨酶（ALT）和天冬氨酸转氨酶（AST）水平升高。ALT和AST与SF水平呈显著正相关，而肾功能检查结果则不然。至于铁螯合剂药物，接受去铁胺治疗的患者（26.5%）的SF水平显著高于接受地拉罗司治疗的患者（73.5%）。本研究强调了建立针对患者的综合评估和随访方案以管理β地中海贫血的重要性，重点是输血和铁螯合治疗措施。
