title: Iron overload in Chinese patients with hemoglobin H disease.
abstract: We examined the iron status of 23 adult patients with hemoglobin H (Hb H) disease. None of them had received multiple blood transfusions or prolonged iron therapy. Studies included serum iron and ferritin concentrations, transferrin saturation, a desferrioxamine test, computed tomography (CT) scan of the liver, and liver biopsy. Iron overload was found in 17 patients (73.9%), especially in males and in patients with splenomegaly (92.9% and 100%, respectively). Four patients with excessive alcohol consumption had clinical manifestations of severe iron overload. Idiopathic hemochromatosis associated HLA antigens, i.e., HLA-A3, -B7, or -B14, were not found in any of the 15 patients tested. These findings indicate that iron overload is common in adult patients with Hb H disease; such patients should abstain from alcohol and be considered for treatment with an iron chelating agent before irreversible organ damage occurs.
authors.affiliation: Division of Hematology, Taipei Veterans General Hospital, Taiwan, Republic of China.
authors.forename: H C
authors.initials: HC
authors.lastname: Hsu
authors.forename: C K
authors.initials: CK
authors.lastname: Lin
authors.forename: S H
authors.initials: SH
authors.lastname: Tsay
authors.forename: E
authors.initials: E
authors.lastname: Tse
authors.forename: C H
authors.initials: CH
authors.lastname: Ho
authors.forename: M P
authors.initials: MP
authors.lastname: Chow
authors.forename: C H
authors.initials: CH
authors.lastname: Yung
authors.forename: H W
authors.initials: HW
authors.lastname: Peng
mesh_terms: D000328:Adult; D000368:Aged; D044466:Asian People; D005260:Female; D005355:Fibrosis; D006447:Hemoglobin H / Q000032:analysis*; D006455:Hemoglobins, Abnormal / Q000032:analysis*; D006801:Humans; D007501:Iron / Q000097:blood*; D008099:Liver / Q000378:metabolism / Q000473:pathology; D008297:Male; D008875:Middle Aged; D013624:Taiwan / Q000208:ethnology; D013789:Thalassemia / Q000097:blood* / Q000208:ethnology
chemical_list: D006455:Hemoglobins, Abnormal; D006447:Hemoglobin H; D007501:Iron