Pathologic Findings -- Hereditary Hemochromatosis


At autopsy, the liver weighed 2850 gm and had a finely nodular appearance. Sectioning revealed a firm, nodular cut surface. The liver, pancreas and lymph nodes all had a distinctly dark brown appearance on sectioning (liver at top, pancreas at bottom center, and lymph nodes at bottom right). The spleen weighed 530 gm. The heart weighed 650 gm and had four chamber dilation. Coronary arteries showed no apparent atherosclerosis. The myocardium was dark brown and firm. The lungs were heavy (2000 gm total) and congested. There was a 1500 cc pleural effusion on the right and a 500 cc effusion on the left. There was 600 cc of ascitic fluid.

Microscopically, with H&E stain the liver had extensive fibrosis with small nodules of hepatocytes without fatty change. Iron stain of liver confirmed the presence of abundant hemosiderin granules. The myocardium with H&E stain showed no evidence of ischemic changes, but there was diffuse intersitital fibrosis, and iron stain of myocardium also demonstrated extensive deposition of hemosiderin in and between myocardial fibers. Additional sites of extensive iron deposition included pancreas, lymph nodes, bone marrow, adrenals, pituitary, testes, thyroid, prostate, trachea, and brain.



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