Kurtoǧllu Ayşegül Uǧur, Koçtekin Belkls, Kurtoǧlu Erdal, Yildiz Mustafa, Bozkurt Selen
Department of Biochemistry, Antalya Education and Research Hospital, Antalya, Turkey.
Department of Tranfusion Center, Antalya Education and Research Hospital, Antalya, Turkey.
Cent Eur J Immunol. 2017;42(1):78-84. doi: 10.5114/ceji.2017.67321. Epub 2017 May 8.
-thalassaemia (-Thal) is considered a severe, progressive haemolytic anaemia, which needs regular blood transfusions for life expectancy. Complement-mediated erythrocyte destruction can cause both intravascular and extravascular haemolysis. Complement regulatory proteins protect cells from such effects of the complement system. We aimed to perform quantitative analysis of membrane-bound complement regulators, CD55 (decay accelerating factor - DAF), CD35 (complement receptor type 1 - CR1), and CD59 (membrane attack complex inhibitory factor - MACIF) on peripheral red blood cells by flow cytometry.
The present study was carried out on 47 -thalassemia major (-TM) patients, 20 -thalassaemia intermedia (-TI) patients, and 17 healthy volunteers as control subjects.
CD55 levels of -TM patients (58.64 ±17.06%) were significantly decreased compared to -TI patients (83.34 ±13.82%) and healthy controls (88.57 ±11.69%) (p < 0.01). CD59 levels of -TM patients were not significantly different than -TI patients and controls, but CD35 levels were significantly lower in the -TM patients (3.56 ±4.87%) and -TI patients (12.48 ±9.19%) than in the control group (39.98 ±15.01%) (p < 0.01).
Low levels of CD55 and CD35 in thalassaemia major patients indicates a role for them in the aetiopathogenesis of haemolysis in this disease, and also this defect in a complement system may be responsible for the chronic complications seen in these patients.
β地中海贫血(β-地贫)被认为是一种严重的、进行性溶血性贫血,患者需要终身定期输血。补体介导的红细胞破坏可导致血管内和血管外溶血。补体调节蛋白可保护细胞免受补体系统的此类影响。我们旨在通过流式细胞术对外周血红细胞上的膜结合补体调节蛋白CD55(衰变加速因子 - DAF)、CD35(1型补体受体 - CR1)和CD59(膜攻击复合物抑制因子 - MACIF)进行定量分析。
本研究对47例重型β地中海贫血(β-TM)患者、20例中间型β地中海贫血(β-TI)患者和17名健康志愿者作为对照进行。
与β-TI患者(83.34 ±13.82%)和健康对照(88.57 ±11.69%)相比,β-TM患者的CD55水平(58.64 ±17.06%)显著降低(p < 0.01)。β-TM患者的CD59水平与β-TI患者和对照组无显著差异,但β-TM患者(3.56 ±4.87%)和β-TI患者(12.48 ±9.19%)的CD35水平显著低于对照组(39.98 ±15.01%)(p < 0.01)。
重型地中海贫血患者CD55和CD35水平较低表明它们在该疾病溶血的病因发病机制中起作用,并且补体系统的这种缺陷可能是这些患者出现慢性并发症的原因。