title: Severe hypercholesterolemia associated with decreased hepatic triglyceride lipase activity and pseudohyponatremia in patients after allogeneic stem cell transplantation.
abstract: A 55-year-old woman with Ph-negative acute lymphoblastic leukemia in primary induction failure received allogeneic peripheral blood stem cell transplantation from her HLA-compatible sister. Pseudohyponatremia developed due to extreme hypercholesterolemia of 4091 mg/dL accompanied by lipoprotein X and lipoprotein Y. The hypercholesterolemia was caused by cholestasis due to chronic GVHD and ischemic cholangiopathy. In addition, we found that hepatic triglyceride lipase (HTGL) activity was severely decreased, which could be another novel factor causing extreme hypercholesterolemia after allogeneic transplantation. The total cholesterol has been gradually decreasing followed by the improvement of cholestasis with bezafibrate, ursodeoxycholic acid and prednisone treatments, and by a slight increase in HTGL-protein. To our knowledge, this is the first report to describe the association of decreased HTGL with extreme hypercholesterolemia after allogeneic transplantation.
authors.affiliation: Department of Hematology, Japanese Red Cross Nagoya First Hospital.
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Inamoto
authors.forename: Tamio
authors.initials: T
authors.lastname: Teramoto
authors.forename: Koji
authors.initials: K
authors.lastname: Shirai
authors.forename: Hideko
authors.initials: H
authors.lastname: Tsukamoto
authors.forename: Takaomi
authors.initials: T
authors.lastname: Sanda
authors.forename: Koichi
authors.initials: K
authors.lastname: Miyamura
authors.forename: Ikuo
authors.initials: I
authors.lastname: Yamamori
authors.forename: Noriyuki
authors.initials: N
authors.lastname: Hirabayashi
authors.forename: Yoshihisa
authors.initials: Y
authors.lastname: Kodera
mesh_terms: D000017:ABO Blood-Group System; D000328:Adult; D001787:Blood Group Incompatibility; D005260:Female; D006801:Humans; D006937:Hypercholesterolemia / Q000209:etiology*; D007010:Hyponatremia / Q000209:etiology*; D008049:Lipase / Q000378:metabolism*; D008099:Liver / Q000201:enzymology*; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000097:blood / Q000628:therapy*; D033581:Stem Cell Transplantation / Q000009:adverse effects*; D014184:Transplantation, Homologous; D016896:Treatment Outcome
chemical_list: D000017:ABO Blood-Group System; D008049:Lipase