Keshk Walaa Arafa, Hablas Nahed Mohammed, Esheba Noha El Sayed, Abd Elsalam Shereen Awny
a Department of Medical Biochemistry and Molecular Biology, Faculty of Medicine , Tanta University , Tanta , Egypt.
b Department of Pediatric, Faculty of Medicine , Tanta University , Tanta , Egypt.
Pediatr Hematol Oncol. 2019 Apr;36(3):151-160. doi: 10.1080/08880018.2019.1607960. Epub 2019 Apr 29.
β-Thalassemia, a blood disease instigated by mutations in β-globin genes or their regulatory regions. Infectious complications, immune abnormalities and iron overload related organ damage are the chief causes of morbidity and mortality. The role of inflammatory cytokines in the pathophysiology of β-thalassemia is still indistinct, so the present study aimed to evaluate immune-inflammatory and redox status in β-thalassemia and their relation to iron status, frequency/duration of blood transfusion. Seventy β-Thalassemia patients (35 β-thalassemia major; 35 β-thalassemia intermedia) and twenty age and sex-matched healthy controls were included. Interleukin4 (IL4), Interleukin10 (IL10), Interleukin 8 (IL8), and Interleukin 13 (IL13) levels were immunoassayed in addition to iron, hematological and redox status assessment. Significantly increased iron, ferritin, IL8, IL13 levels ( value ˂0.001), meanwhile, decreased IL10 level, total anti-oxidant (TAC) and iron binding capacities were observed in β-thalassemia major compared with β-thalassemia intermedia and control groups ( value ˂0.001) . IL4 level was decreased in patients group compared to control. Positive correlation was found between malondialdehyde (MDA), IL8, IL13 and iron load, transfusion frequency/duration, total leucocyte count and lymphocyte (%). Meanwhile, negative correlation was found with IL4, IL10, and TAC. Multiple blood transfusions increased iron stores which may critically impair the immune-regulatory balance with disturbed redox status and cytokine profile.
β地中海贫血是一种由β珠蛋白基因或其调控区域突变引发的血液疾病。感染性并发症、免疫异常以及铁过载相关的器官损害是发病和死亡的主要原因。炎症细胞因子在β地中海贫血病理生理学中的作用仍不明确，因此本研究旨在评估β地中海贫血患者的免疫炎症和氧化还原状态，以及它们与铁状态、输血频率/持续时间的关系。研究纳入了70例β地中海贫血患者（35例重型β地中海贫血；35例中间型β地中海贫血）以及20例年龄和性别匹配的健康对照者。除了评估铁、血液学和氧化还原状态外，还采用免疫分析法检测了白细胞介素4（IL4）、白细胞介素10（IL10）、白细胞介素8（IL8）和白细胞介素13（IL13）的水平。与中间型β地中海贫血组和对照组相比，重型β地中海贫血组的铁、铁蛋白、IL8、IL13水平显著升高（P值˂0.001），同时IL10水平、总抗氧化能力（TAC）和铁结合能力降低（P值˂0.001）。与对照组相比，患者组的IL4水平降低。丙二醛（MDA）、IL8、IL13与铁负荷、输血频率/持续时间、白细胞总数和淋巴细胞百分比呈正相关。同时，与IL4、IL10和TAC呈负相关。多次输血会增加铁储存，这可能会严重损害免疫调节平衡，导致氧化还原状态和细胞因子谱紊乱。
