Ibrahim Hamdy A, Fouda Manal I, Yahya Raida S, Abousamra Nashwa K, Abd Elazim Rania A
Department of Physiology, Faculty of Science, Mansoura University, Egypt.
Department of Clinical Pathology, Faculty of Medicine, Mansoura University, Egypt.
Lab Hematol. 2014 Jun;20(2):9-14. doi: 10.1532/LH96.12016.
[ABS]Phospholipid asymmetry is well maintained in erythrocyte (RBC) membranes with phosphatidylserine (PS) exclusively present in the inner leaflet. Eryptosis, the suicidal death of RBCs, is characterized by cell shrinkage, membrane blebbing, and cell membrane phospholipids scrambling with PS exposure at the cell surface. Erythrocytes exposing PS are recognized, bound, engulfed, and degraded by macrophages. Eryptosis thus fosters clearance of affected RBCs from circulating blood, which may aggravate anemia in pathological conditions. Thalassemia patients are more sensitive to the eryptotic depletion and osmotic shock which may affect RBC membrane phospholipid asymmetry. We aimed in this work to determine the RBC PS exposure in splenectomized and nonsplenectomized β-thalassemia major (β-TM) patients and correlate it with the clinical presentation and laboratory data. RBCs were stained for annexin V to detect phosphatidylserine (PS) exposure in 46 β-TM patients (27 splenectomized and 19 nonsplenectomized) compared to 17 healthy subjects as a control group. We observed a significant increase in RBC PS exposure in β-TM patients compared to control group (P = .0001). Erythrocyte PS exposure was significantly higher in splenectomized β-TM patients compared with nonsplenectomized β-TM patients (P = .001). No correlation was found between RBC PS exposure and clinical or hematological data of β-TM patients, but there was a positive correlation between RBC PS exposure and ferritin level in β-TM patients have higher levels of RBC PS exposure, and splenectomy was shown to aggravate RBC PS exposure without aggravation of anemia.
[摘要] 磷脂不对称性在红细胞(RBC)膜中得到良好维持,磷脂酰丝氨酸(PS)仅存在于内膜层。红细胞凋亡,即红细胞的自杀性死亡,其特征为细胞收缩、膜泡形成以及细胞膜磷脂紊乱,导致PS暴露于细胞表面。暴露PS的红细胞会被巨噬细胞识别、结合、吞噬并降解。因此,红细胞凋亡促进了循环血液中受影响红细胞的清除,这在病理状态下可能会加重贫血。地中海贫血患者对红细胞凋亡性消耗和渗透休克更为敏感,这可能会影响RBC膜磷脂的不对称性。我们在这项研究中的目的是确定脾切除和未脾切除的重型β地中海贫血(β-TM)患者的RBC PS暴露情况,并将其与临床表现和实验室数据相关联。与17名健康受试者作为对照组相比,对46名β-TM患者(27名脾切除和19名未脾切除)的红细胞进行膜联蛋白V染色,以检测磷脂酰丝氨酸(PS)暴露情况。我们观察到,与对照组相比,β-TM患者的RBC PS暴露显著增加(P = .0001)。脾切除的β-TM患者的红细胞PS暴露显著高于未脾切除的β-TM患者(P = .001)。在β-TM患者的RBC PS暴露与临床或血液学数据之间未发现相关性,但在β-TM患者中,RBC PS暴露与铁蛋白水平呈正相关,β-TM患者的RBC PS暴露水平较高,并且脾切除术显示会加重RBC PS暴露而不会加重贫血。