Zidan Hanaa, Lo Stanley, Wiebe Donald, Talano Julie, Alemzadeh Ramin
Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
Pediatr Blood Cancer. 2008 Jun;50(6):1280-1. doi: 10.1002/pbc.21522.
We describe a case of extreme hypercholesterolemia, mediated by lipoprotein X, in a 12-year-old Caucasian female who underwent an unrelated allogenic bone marrow transplant for relapsed acute myelocytic leukemia (AML). Her post-transplant course was complicated by severe chronic graft-versus-host disease (GVHD) of the liver. Previously normal serum cholesterol and triglycerides rose to 1,122 mg/dl (29.0 mmol/L) and 1,100 mg/dl (12.4 mmol/L), respectively. Serum cholesterol appeared to be dominantly carried by lipoprotein X. Intra-hepatic cholestasis leading to reflux of bile lipoproteins into the blood stream and subsequent formation of lipoprotein X appears to be the mechanism underlying this phenomenon.
我们描述了一例由脂蛋白X介导的极端高胆固醇血症病例，患者为一名12岁的白种女性，因复发性急性髓细胞白血病（AML）接受了非亲缘异基因骨髓移植。她移植后的病程因严重的慢性肝移植物抗宿主病（GVHD）而复杂化。之前正常的血清胆固醇和甘油三酯分别升至1122mg/dl（29.0mmol/L）和1100mg/dl（12.4mmol/L）。血清胆固醇似乎主要由脂蛋白X携带。肝内胆汁淤积导致胆汁脂蛋白反流进入血流，随后形成脂蛋白X，这似乎是这一现象的潜在机制。
