Severe hypercholesterolemia mediated by lipoprotein X in a pediatric patient with chronic graft-versus-host disease of the liver.
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.
Zidan
Hanaa
H
Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
Lo
Stanley
S
Wiebe
Donald
D
Talano
Julie
J
Alemzadeh
Ramin
R
D001655:Bile Reflux / Q000209:etiology; D002648:Child; D002780:Cholestasis, Intrahepatic / Q000209:etiology*; D002908:Chronic Disease; D005260:Female; D006086:Graft vs Host Disease / Q000097:blood* / Q000209:etiology; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects*; D006801:Humans; D006937:Hypercholesterolemia / Q000097:blood* / Q000209:etiology; D008073:Lipoprotein-X / Q000097:blood*
D008073:Lipoprotein-X