Re: hemochromatosis

Posted By HFHSM.D.-rf on July 29, 1998 at 20:42:46:

In Reply to: hemochromatosis posted by Adam L. on July 24, 1998 at 10:33:22:






    My grandfather has leukemia and, consequently, has to have
blood transfusions every five to six weeks. He recently became
very sick and was diagnosed with hemochromatosis. What exactly is
hemochromatosis  and what are the most effective procedures
in dealing with the disease?  
                                  Thank you,
                                    Adam L.
__


Dear Adam L,
Hemochromatosis is an inherited disease in which excessive iron is absorbed form the gastrointestinal tract.  Iron is deposited in the liver, heart, skin, pancreas and other organs causing impaired function and scarring.  If a family member is diagnosed with hemochromatosis, it is prudent to screen relatives because of the possibility that they too have the disease.
Individuals who are receiving frequent blood transfusions (like your grandfather) can have excessibe deposition of iron in the liver because the transfused red blood cells have a shorter survivial and when they break down iron is deposited in the liver.  This condition which is not inherited is called hemosiderosis.  Family members are not at increased risk of hemosiderosis.
A pathologist with experience in liver disease should be able to distinguish hemochromatosis from hemosiderosis.  In hemochromatosis iron is deposited in the liver cells (hepatocytes), whereas in hemosiderosis iron is deposited in another cell type.  Ask the pathologist to review the liver biopsy to verify the diagnosis.
This information is provided for educational purposes only.  Always ask specific questions to your personal physician.
HFHSM.D.-rf
*keywords: hemochromatosis, hemosiderosis, iron
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