Abstract
Background: β-thalassaemia (β-Thal) is an inherited chronic haemolytic anaemia resulting from absent or low level of synthesis of β-globin chains of haemoglobin A in erythropoietic cells. The complement system is an important part of innate immune response that may be implicated in red blood cell (RBC) lysis. Mammalian cells are provided with surface bound complement regulatory proteins (MCRPs) that regulate the activation of complement cascade, thus protecting them from uncontrolled complement-mediated lysis. Objective is to evaluate the role of complement regulatory proteins (CD55, CD59, and CD35) on red blood cells, and to explain the pathogenesis of anaemia in β-thalassemia major. Methods: This case-control study enrolled 74 β thalassemia major patients who were compared with 40 age and sex matched controls. We performed expression of CD55, CD59, and CD35 on RBCs using flow cytometry. Results: CD55 levels of β-thalassemia major patients (79.78% ± 18.54%) were significantly decreased compared to healthy controls (99.45% ± 0.59%) (P β-thalassemia major patients (97.76% ± 1.72%) were significantly lower than in the controls (99.75% ± 0.36%) (P β-thalassemia major patients (4.30% ± 4.66%) than in the control group (19.40% ± 10.90%) (P Conclusion: β-thalassemia major patients suffer from increased haemolysis and a consequent increase in their demand for blood transfusion. Complement-mediated haemolysis was shown in our study by decreased expression of CD55, CD59, and CD35 in β-thalassemia major patients. This allows complement deposition on RBCs and enhances or accelerates their lysis.
Highlights
Background: β-thalassaemia (β-Thal) is an inherited chronic haemolytic anaemia resulting from absent or low level of synthesis of β-globin chains of haemoglobin A in erythropoietic cells
The thalassemias are a group of inherited disorders that are caused by altered or absent hemoglobin chain synthesis leading to ineffective erythropoiesis and subsequent anemia
We found a high significant difference between studied groups as regards CD55%, CD59%, and CD35% on red blood cell (RBC) with significantly decreased expression of CD55%, CD59%, and CD35% on RBCs were found among patients compared to controls
Summary
Background: β-thalassaemia (β-Thal) is an inherited chronic haemolytic anaemia resulting from absent or low level of synthesis of β-globin chains of haemoglobin A in erythropoietic cells. The complement system is an important part of innate immune response that may be implicated in red blood cell (RBC) lysis. Objective is to evaluate the role of complement regulatory proteins (CD55, CD59, and CD35) on red blood cells, and to explain the pathogenesis of anaemia in β-thalassemia major. Complement-mediated haemolysis was shown in our study by decreased expression of CD55, CD59, and CD35 in β-thalassemia major patients. This allows complement deposition on RBCs and enhances or accelerates their lysis. Though complement is an essential part of the immune response against microbes, the complement system plays crucial roles in maintaining homeostasis through such mechanisms as the removal of apoptotic cells, the regulation of coagulation, angiogenesis, and lipid metabolism and, importantly, the surveillance of neoplastic cells [6]
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