Severe hypercholesterolemia mediated by lipoprotein X in patients with chronic graft-versus-host disease of the liver. Bone Marrow Transplant 2005 Jan;35(1):85-9
Date
11/09/2004Pubmed ID
15531904DOI
10.1038/sj.bmt.1704739Scopus ID
2-s2.0-11844255681 (requires institutional sign-in at Scopus site) 44 CitationsAbstract
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.
Author List
Turchin A, Wiebe DA, Seely EW, Graham T, Longo W, Soiffer RAuthor
Walter L. Longo MD Professor in the Medicine department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
AdultBone Marrow Transplantation
Cholestasis
Cholesterol
Female
Graft vs Host Disease
Humans
Hypercholesterolemia
Lipoprotein-X
Liver
Liver Diseases
Male
Middle Aged
Time Factors
Transplantation, Homologous