Ginsberg H, Gilbert H S, Gibson J C, Le N A, Brown W V
Ann Intern Med. 1982 Mar;96(3):311-6. doi: 10.7326/0003-4819-96-3-311.
Hypocholesterolemia reported in patients with myeloproliferative disorders prompted our investigation of lipoprotein metabolism in these patients. The production and fractional catabolic rates of very-low-density lipoprotein (VLDL) apoprotein-B were measured using 131I-VLDL; those of VLDL triglyceride, using 3H-glycerol; and those of low-density lipoprotein (LDL) apoprotein-B, using 125I-LDL. Plasma total and LDL cholesterol levels (mean +/- SD) were significantly reduced in seven patients with myeloproliferative diseases, compared to five normal subjects (93.1 +/- 20.3 mg/dL versus 166.8 +/- 24.6 mg/dL and 50.3 +/- 14.8 mg/dL versus 107 +/- 20.8 mg/dL, respectively). The production rates of VLDL apoprotein-B and VLDL triglyceride were normal in the patients. The fractional catabolic rate of LDL apoprotein-B was increased in the patients with myeloproliferative diseases (0.89 +/- 0.32/d versus 0.52 +/- 0.10/d; p less than 0.05); this increased rate was associated with reduced plasma LDL apoprotein-B levels (41.7 +/- 7.1 mg/dL versus 57.0 +/- 11.3 mg/dL; p less than 0.05) despite normal or elevated LDL apoprotein-B production (16.7 +/- 5.3 mg/kg body weight . d versus 12.9 +/- 1.2 mg/kg body weight . d). The site (or sites) of increased LDL catabolism in these hypocholesterolemic patients with myeloproliferative disorders is under investigation.
骨髓增殖性疾病患者中报告的低胆固醇血症促使我们对这些患者的脂蛋白代谢进行研究。使用¹³¹I-VLDL测量极低密度脂蛋白（VLDL）载脂蛋白B的生成率和部分分解代谢率；使用³H-甘油测量VLDL甘油三酯的生成率和部分分解代谢率；使用¹²⁵I-LDL测量低密度脂蛋白（LDL）载脂蛋白B的生成率和部分分解代谢率。与五名正常受试者相比，七名骨髓增殖性疾病患者的血浆总胆固醇和LDL胆固醇水平（均值±标准差）显著降低（分别为93.1±20.3mg/dL对166.8±24.6mg/dL和50.3±14.8mg/dL对107±20.8mg/dL）。患者中VLDL载脂蛋白B和VLDL甘油三酯的生成率正常。骨髓增殖性疾病患者中LDL载脂蛋白B的部分分解代谢率增加（0.89±0.32/d对0.52±0.10/d；p<0.05）；尽管LDL载脂蛋白B生成正常或升高（16.7±5.3mg/kg体重·d对12.9±1.2mg/kg体重·d），但该增加的速率与血浆LDL载脂蛋白B水平降低相关（41.7±7.1mg/dL对57.0±11.3mg/dL；p<0.05）。这些低胆固醇血症的骨髓增殖性疾病患者中LDL分解代谢增加的部位正在研究中。
