Hemochromatosis

HEMOCHROMATOSIS

• Hemochromatosis is a common inherited disorder of iron metabolism leading to deposition of excessive amount of iron in parenchymal cells leading to fibrosis and organ failure.
• The iron storage pigment in tissues is called as hemosiderin (least in skin).
• Hemosiderosis is an acquired condition.
• Liver is the first organ to be affected & maximum deposition of iron by hemochromatosis.
• Classic triad is- cirrhosis, diabetes mellitus, skin pigmentation.

Conditions of Iron Overload States– classified as

I) Herediatry- hemochromatosis

II) Acquired overload-

a) Iron loading-

1. Thalassemia major
2. Sideroblastic anaemia
3. Chronic haemolytic anaemia

IRON POISONING

• Ingestion of a number of ferrous sulphate tablets may cause acute iron poisoning.
• Clinical features : Severe vomiting ,Diarrhea ,GI bleeding ,there may be severe shock, hepatic & renal failure
• Antidotes:Desferrioxamine is given parenterally (IM or IV), whereas deferiprone and deferasirox are used orally.