Graft-Versus-Host Disease

Bone Marrow Transplantation (2005) 35, 85–89. doi:10.1038/sj.bmt.1704739 Published online 8 November 2004

Severe hypercholesterolemia mediated by lipoprotein X in patients with chronic graft-versus-host disease of the liver

A Turchin1, D A Wiebe2, E W Seely1, T Graham3, W Longo4 and R Soiffer5

  1. 1Division of Endocrinology, Diabetes and Hypertension, Brigham and Women's Hospital, Boston, MA, USA
  2. 2Department of Pathology and Laboratory Medicine, University of Wisconsin, Madison, WI, USA
  3. 3Division of Endocrinology, Beth Israel Deaconess Medical Center, Boston, MA, USA
  4. 4Division of Hematology, University of Wisconsin, Madison, WI, USA
  5. 5Department of Medical Oncology, Dana Farber Cancer Institute, Boston, MA, USA

Correspondence: Dr A Turchin, Division of Endocrinology, Diabetes and Hypertension, Brigham and Women's Hospital, 221 Longwood Avenue, Boston, MA 02115, USA. E-mail: aturchin@partners.org

Received 18 May 2004; Accepted 27 August 2004; Published online 8 November 2004.

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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.

Keywords:

hyperlipidemia, hypercholesterolemia, graft-versus-host disease, lipoprotein X

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