Naoum Flávio A, Gualandro Sandra F M, Latrilha Maria da Conceição M, Maranhão Raul C
Hematology Service, University of São Paulo Medical School, São Paulo, Brazil.
Am J Hematol. 2004 Dec;77(4):340-5. doi: 10.1002/ajh.20206.
Patients with beta-thalassemia trait have been reported to present lower plasma concentrations of low-density lipoprotein (LDL) and lower frequencies of acute myocardial infarction than normal subjects. In this study, the metabolism of LDL was tested in 12 patients with heterozygous beta-thalassemia trait (HBT) and 13 healthy subjects without the disease by determining the plasma kinetics of an artificially made cholesterol-rich microemulsion (LDE) that mimics the LDL metabolism and binds to LDL receptors. The emulsion was labeled with 14C-cholesterol ester and injected intravenously into the subjects. Blood samples were drawn at regular intervals over 24 hr to determine the plasma decay curve of the emulsion radioactive label and to estimate its plasma fractional clearance rate (FCR, in hr(-1)). FCR of the 14C-cholesterol ester was greater in HBT compared to controls (0.0631 +/- 0.0178 hr(-1) and 0.0501 +/- 0.0094 hr(-1), respectively; mean +/- SD, P = 0.022). No differences were found regarding LDL cholesterol plasma concentration between the two groups, but apolipoprotein B concentration was lower in HBT than in control subjects (80 +/- 44 and 96 +/- 14, respectively; mean +/- SD, P = 0.026). Our results show that LDE FCR is increased in HBT, indicating that LDL clearance is increased in patients with beta-thalassemia trait possibly due to the increased proliferation in the bone marrow of erythroid precursors.
据报道,与正常受试者相比,β地中海贫血特征患者的血浆低密度脂蛋白(LDL)浓度较低,急性心肌梗死发生率也较低。在本研究中,通过测定一种人工合成的富含胆固醇的微乳剂(LDE)的血浆动力学来检测12例杂合子β地中海贫血特征(HBT)患者和13例无此病的健康受试者的LDL代谢,该微乳剂模拟LDL代谢并与LDL受体结合。该乳剂用14C - 胆固醇酯标记,并静脉注射到受试者体内。在24小时内定期采集血样,以确定乳剂放射性标记的血浆衰减曲线,并估计其血浆分数清除率(FCR,单位为小时-1)。与对照组相比,HBT患者中14C - 胆固醇酯的FCR更高(分别为0.0631±0.0178小时-1和0.0501±0.0094小时-1;均值±标准差,P = 0.022)。两组之间LDL胆固醇血浆浓度无差异,但HBT患者的载脂蛋白B浓度低于对照组(分别为80±44和96±14;均值±标准差,P = 0.026)。我们的结果表明,HBT患者的LDE FCR增加,这表明β地中海贫血特征患者的LDL清除增加,可能是由于红系前体细胞在骨髓中的增殖增加所致。