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Histological changes in cardiac hemochromatosis improved by an iron-chelating agent. A biopsy case
A Tashiro1, R Satodate, I Segawa
1Department of Pathology II, Iwate Medical University School of Medicine, Morioka, Japan.
Insights
Deferoxamine mesylate improved cardiac dysfunction in a patient with secondary hemochromatosis. Iron chelation therapy reduced hemosiderin in heart muscle, but liver iron remained.
Area of Science:
- Cardiology
- Hematology
- Pathology
Background:
- Secondary hemochromatosis can cause cardiac dysfunction and ECG abnormalities.
- Sideroblastic anemia is a potential underlying condition associated with iron overload.
Observation:
- A 51-year-old woman presented with cardiac dysfunction and ECG abnormalities.
- Endomyocardial biopsy revealed hemosiderin deposition in myocytes, causing vacuolization and disarray.
Findings:
- Treatment with deferoxamine mesylate, an iron-chelating agent, led to clinical improvement.
- Repeat endomyocardial biopsy showed reduced hemosiderin, improved myocyte vacuolization, and disarray.
- Ultrastructural analysis and X-ray microanalysis confirmed decreased iron granules within myocytes.
Implications:
- Deferoxamine mesylate is effective in treating cardiac siderosis secondary to hemochromatosis.
- While cardiac iron can be reduced, persistent liver iron deposition requires further monitoring.
- This case highlights the importance of evaluating cardiac function in patients with iron overload disorders.
Abstract:
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.