Liver injury due to iron overload in thalassemia: histopathologic and ultrastructural studies.
The livers of 30 cases of thalassemia (19/beta-thal/HbE, seven thal/HbH and four beta-thal major) were studied histopathologically and electron microscopically, in an effort to define the morphologic alterations due to iron overload. The results of light and electron microscopy were similar in most cases. Iron accumulation and fibrosis were the common features found in these patients, except that thal/HbH exhibited lesser hepatic damage. The degrees of iron deposition and fibrosis were found to be higher in splenectomized and cirrhotic than non-splenectomized and non-cirrhotic patients. The subcellular changes were swollen mitochondria, with the presence of an electron dense matrix and ruptured mitochondrial membrane. Proliferation of smooth endoplasmic reticulum (ER) and dilated rough ER was observed. Increases in lysosomal hemosiderin in hepatocytes and in Kupffer cells were demonstrated. The possible ways by which the iron compounds or free radicals mediated membrane damage are mentioned. The pattern of liver cell damage is similar to that of viral hepatitis.
Thakerngpol
K
K
Department of Pathology, Mahidol University, Bangkok, Thailand.
Fucharoen
S
S
Boonyaphipat
P
P
Srisook
K
K
Sahaphong
S
S
Vathanophas
V
V
Stitnimankarn
T
T
D000293:Adolescent; D000328:Adult; D001803:Blood Transfusion; D003094:Collagen / Q000032:analysis; D005260:Female; D006454:Hemoglobins / Q000032:analysis; D006485:Hemosiderin / Q000032:analysis; D006801:Humans; D007501:Iron / Q000032:analysis / Q000633:toxicity*; D008099:Liver / Q000187:drug effects / Q000473:pathology* / Q000648:ultrastructure; D008247:Lysosomes / Q000473:pathology; D008297:Male; D008930:Mitochondria, Liver / Q000187:drug effects / Q000473:pathology / Q000648:ultrastructure; D008933:Mitochondrial Swelling; D012044:Regression Analysis; D013156:Splenectomy; D017085:alpha-Thalassemia / Q000473:pathology* / Q000628:therapy; D017086:beta-Thalassemia / Q000473:pathology* / Q000628:therapy
D006454:Hemoglobins; D003094:Collagen; D006485:Hemosiderin; D007501:Iron