Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018133 (graft-versus-host disease)
 18,032 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 22-year-old female with diffuse mixed T cell lymphoma in second complete remission underwent allogeneic BMT from her HLA-compatible brother. Transplantation was complicated by acute graft-versus-host disease (GVHD), cytomegalovirus (CMV) infection, and combined hepatotoxic/cholestatic liver disease 45 days post-BMT. Cholesterol levels reached 65 mmol/l, and high density lipoprotein (HDL) cholesterol decreased to 0.23 mmol/l. She developed skin xanthelasmas, lipemia retinalis, and a solitary lung lesion, which was clinically diagnosed as pulmonary cholesteroloma. All these complications resolved following plasmapheresis and hypolipidemic treatment with lovastatin and cholesterol levels normalized.
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PMID:Solitary pulmonary cholesteroloma, multiple xanthelasmas and lipemia retinalis complicating hypercholesterolemia after bone marrow transplantation. 886 64

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.
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PMID:Severe hypercholesterolemia mediated by lipoprotein X in patients with chronic graft-versus-host disease of the liver. 1553 4