Iron overload in Chinese patients with hemoglobin H disease.
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.
Hsu
H C
HC
Division of Hematology, Taipei Veterans General Hospital, Taiwan, Republic of China.
Lin
C K
CK
Tsay
S H
SH
Tse
E
E
Ho
C H
CH
Chow
M P
MP
Yung
C H
CH
Peng
H W
HW
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
D006455:Hemoglobins, Abnormal; D006447:Hemoglobin H; D007501:Iron