title: Severe hypercholesterolemia mediated by lipoprotein X in patients with chronic graft-versus-host disease of the liver.
abstract: We describe a series of cases of extreme hypercholesterolemia mediated by lipoprotein X in patients with chronic graft-versus-host disease of the liver after an allogeneic bone marrow transplant. All of the patients presented with a total cholesterol in excess of 1000 mg/dl (25.9 mmol/l). At the time they were also noted to have pseudohyponatremia. Cholesterol appeared to be predominantly carried by lipoprotein X. Intrahepatic cholestasis leading to reflux of bile lipoproteins into the bloodstream and subsequent formation of protein X appears to be the mechanism underlying this phenomenon. Complications, including retinal cholesterol thromboembolism and cholesteroloma of the lung have been seen in the patient with the highest cholesterol levels. Severe hypercholesterolemia is an important, and likely more common than previously reported, long-term complication of allogeneic hematopoietic stem cell transplantation. It is important for clinicians to familiarize themselves with the diagnostic and therapeutic challenges this condition presents.
authors.affiliation: Division of Endocrinology, Diabetes and Hypertension, Brigham and Women's Hospital, 221 Longwood Avenue, Boston, MA 02115, USA. aturchin@partners.org
authors.forename: A
authors.initials: A
authors.lastname: Turchin
authors.forename: D A
authors.initials: DA
authors.lastname: Wiebe
authors.forename: E W
authors.initials: EW
authors.lastname: Seely
authors.forename: T
authors.initials: T
authors.lastname: Graham
authors.forename: W
authors.initials: W
authors.lastname: Longo
authors.forename: R
authors.initials: R
authors.lastname: Soiffer
mesh_terms: D000328:Adult; D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D002779:Cholestasis; D002784:Cholesterol / Q000097:blood; D005260:Female; D006086:Graft vs Host Disease / Q000150:complications*; D006801:Humans; D006937:Hypercholesterolemia / Q000150:complications / Q000378:metabolism*; D008073:Lipoprotein-X / Q000502:physiology*; D008099:Liver / Q000473:pathology; D008107:Liver Diseases / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D013997:Time Factors; D014184:Transplantation, Homologous / Q000009:adverse effects
chemical_list: D008073:Lipoprotein-X; D002784:Cholesterol