Histological changes in cardiac hemochromatosis improved by an iron-chelating agent. A biopsy case.
Cardiac dysfunction and ECG abnormalities were demonstrated in a 51-year-old woman suffering from secondary hemochromatosis in sideroblastic anemia. Hemosiderin deposition in vacuolized and disarrayed myocytes was disclosed by microscopic examination of the first biopsy specimen of endomyocardial tissue. Three months after administration of deferoxamine mesylate, an iron-chelating agent, the clinical findings were improved. The second endomyocardial biopsy revealed marked depletion of hemosiderin deposition in the myocytes, and improvement of myocyte vacuolization and disarray. Ultrastructurally, the highly electron-dense granules in the myocytes were also decreased in number and density. X-ray microanalysis revealed a prominent peak of Fe in the granules. In a liver specimen obtained by needle biopsy 5 months after the second endomyocardial biopsy, marked hemosiderin deposition still remained.
Tashiro
A
A
Department of Pathology II, Iwate Medical University School of Medicine, Morioka, Japan.
Satodate
R
R
Segawa
I
I
D001706:Biopsy; D009202:Cardiomyopathies / Q000188:drug therapy / Q000473:pathology*; D003300:Copper / Q000032:analysis; D003594:Cytoplasmic Granules / Q000032:analysis / Q000648:ultrastructure; D004577:Electron Probe Microanalysis; D005260:Female; D006432:Hemochromatosis / Q000188:drug therapy / Q000473:pathology*; D006485:Hemosiderin / Q000032:analysis; D006801:Humans; D007501:Iron / Q000032:analysis; D007502:Iron Chelating Agents / Q000627:therapeutic use*; D008099:Liver / Q000473:pathology; D008854:Microscopy, Electron; D008875:Middle Aged; D009206:Myocardium / Q000032:analysis / Q000473:pathology / Q000648:ultrastructure; D009992:Osmium / Q000032:analysis
D007502:Iron Chelating Agents; D009992:Osmium; D003300:Copper; D006485:Hemosiderin; D007501:Iron