Later, fibrous septae and regenerative nodules typical of cirrhosis develop. Initially, the most obvious lesion in hemochromatosis is the presence of brown pigment, particularly in the cells in the periportal area. Staining for iron reveals characteristic bluish pigmentation of these cells. Since iron accumulates only in the hepatocytes, the Kupfer cells and endothelial cells are not affected. Chronic liver injury results in liver cell death and the development of a fine, micronodular cirrhosis.
Treatment involves the use of chelators of iron to remove the excessive iron pool in the body.
With the realization that hemochromatosis is an inherited disorder, there is the possibility of HLA typing to identify HLA haplotypes associated with hemochromatosis. This should provide information for genetic counseling of affected individuals. Robbins Pathologic Basis of Disease. Search Medline at National Library of Medicine Please be patient during transfer. Medline will open in a new window.