Allen D W, Manning N
Department of Veterans Affairs Medical Center, Minneapolis, MN 55417.
Blood. 1994 Aug 15;84(4):1283-7.
Spur cell anemia is a hemolytic anemia seen in severe alcoholic cirrhosis that is characterized by unusual morphology and a decreased ratio of phospholipids to cholesterol in the erythrocyte membrane. We hypothesized that defective phospholipid repair may contribute to the red blood cell (RBC) phospholipid abnormalities of spur cell anemia. Therefore, we compared RBCs from normal control subjects with RBCs from spur cell anemia patients. The incorporation of [14C] arachidonic acid into the phospholipids and acylcarnitine (acyl-Cn) of spur cells and normal RBCs was analyzed by a direct-phase high performance liquid chromatography column to separate both the phospholipids and acyl-Cn. There was less uptake of the [14C] arachidonate into phosphatidylethanolamine of spur cell RBCs (12.9% +/- 1.0%) compared with normal RBCs (20.5% +/- 2.8%; P = .0245). However, more arachidonate was incorporated into the acyl-Cn of spur cells (spur cell acyl-Cn [24.5% +/- 2.9%] v normal control acyl-Cn [10.1% +/- 1.9%]; P = .0018). We conclude that phospholipid biosynthesis is inhibited and that acyl-Cn formation is spared in spur cell anemia RBCs. These metabolic changes may help account for the lipid abnormalities seen in spur cell anemia RBCs and contribute to the hemolytic process.
棘状细胞贫血是一种在严重酒精性肝硬化中出现的溶血性贫血,其特征是形态异常以及红细胞膜中磷脂与胆固醇的比例降低。我们推测磷脂修复缺陷可能导致棘状细胞贫血的红细胞(RBC)磷脂异常。因此,我们将正常对照受试者的红细胞与棘状细胞贫血患者的红细胞进行了比较。通过直接相高效液相色谱柱分析[14C]花生四烯酸掺入棘状细胞和正常红细胞的磷脂及酰基肉碱(acyl-Cn)中的情况,以分离磷脂和酰基-Cn。与正常红细胞(20.5%±2.8%)相比,棘状细胞贫血患者的红细胞对[14C]花生四烯酸摄取到磷脂酰乙醇胺中的量较少(12.9%±1.0%;P = 0.0245)。然而,更多的花生四烯酸掺入到棘状细胞的酰基-Cn中(棘状细胞酰基-Cn [24.5%±2.9%]对正常对照酰基-Cn [10.1%±1.9%];P = 0.0018)。我们得出结论,在棘状细胞贫血的红细胞中磷脂生物合成受到抑制,而酰基-Cn的形成未受影响。这些代谢变化可能有助于解释棘状细胞贫血红细胞中所见的脂质异常,并促进溶血过程。