Transfusional hemochromatosis: Classifications and clinical manifestations

Hemochromatosis is a condition caused by extraordinary iron deposition in parenchymal cells leading to cellular damage and organ dysfunction. It is also called iron overload.

The disorder has two classifications:
*Genetic (or primary) hemochromatosis and
*Secondary (or acquired) hemochromatosis.

In secondary hemochromatosis, it may occur as a result of other blood disorders, such as thalassemia or certain anemias. Too many blood transfusions over time may lead to iron overload.

Transfusional hemosiderosis is the accumulation of iron in the liver and heart but also endocrine organs, in patients who receive or did receive frequent blood transfusions (such as those with thalassemia, sickle cell disease, leukemia, aplastic anemia or myelodysplastic syndrome).

𝛽-thalassemia major is one of the causes of secondary hemochromatosis with adoption of an intensive transfusional regimen to maintain adequate levels of hemoglobin.

Cirrhosis and diabetes are known clinical manifestations in patients with transfusional hemochromatosis. A unit of transfused blood contains approximately 250 mg of iron. The excess iron from the transfused erythrocytes gradually accumulates in various tissues, in the skin, heart, gonads, and endocrine glands causing morbidity and mortality.
Transfusional hemochromatosis: Classifications and clinical manifestations