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Terms in this set (8)
What is 'iron over load'
• This is a pathological state in which total body stores or iron are increased.
• This can often result in organ dysfunction as a result of iron deposition
List causes of iron overload
• Primary hereditary hemochromatosis
o Autosomal recessive inheritance
o Association with excessive iron absorption
o Mostly due to a homozygous mutation in the HFE gene on chromosome 6
o Can be due to a mutation of the hepcidin or haemojuvelin genes
o All causes show low levels of hepcidin
• African iron overload
o Dietary and genetic components
• Excess dietary iron
• Ineffective erythropoiesis with increased iron absorption due to inappropriately low levels hepcidin
• Repeated blood transfusions in patients with severe refractory anaemia (Such as thal major or myelodysplasia
• Each unit of blood contains 250mg or iron
List some clinical features of iron overload
• These are mainly caused by organ dysfunction as a result of iron deposition
o Arrhythmias and congestive heart failure
• Growth/sexual development reduced in children
• Liver showing cirrhosis and haemosiderosis
• Excessive melanin skin pigmentation
• Excessive infections
• Arthropathy in genetic hemochromatosis caused by pyrophosphate deposition
Give some lab features for iron overload
• Serum iron and transferrin saturation high
• Raised serum ferritin
• Increased iron in the liver
• Abnormal LFTs
• Increased urinary iron excretion in response to iron chelation therapy
• Cardiomyopathies causing weird ECGs
• MRI can detect increased iron in heart and liver
• Endocrine abnormalities (Raised blood glucose)
General treatment for iron overload
• Genetic hemochromatosis
o Regular venesections to reduce iron to normal
o Assess need by using serum ferritin, iron and TIBC
• Tranfusional iron overload
o Iron chelation therapy
How does iron poisoning present?
Also include acute treatment of iron poisoning
• Clinically presents with abdominal pain, constipation, anaemia and peripheral neuropathy with a blue, lead, line in the gums.
• The blood film will show punctate basophilia (Blue staining as a result of staining of the RNA) as well as features of haemolysis. May also show ringed sideroblasts
• Commonest in childhood and usually accidental
• Clinically, nausea, abdo pain, diarrhoea, GI bleeding and odd LFTs
• Immediate treatment with a gastric lavage with ?whole bowel irrigation.
• IV desferrioxamine is valuable in chelating iron and reducing the risk of liver damage.
What is iron chelation therapy?
• Iron overload can be treated with phlebotomy or iron chelation therapy
• Iron chelation is a drug therapy for iron overload.
• The drugs are called 'chelators' that remove extra iron in your body - and put it back down to safe levels
• Examples are Deferoxamine and Deferasirox
Go ovber sideroblastic anaemia
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