Iron Overload in Thalassaemic Patients

It it a need for patients with thalassaemia to be transfused with blood at least once a month especially when they are of thalassaemia major which is the severe form that need blood tranfusion.

And, sadly to say; there is no medication in this world that can be given without any side effect. Though blood tranfusion helped them to prolonged their life as campared to those in the ancient time, it brought up another problem for them - Iron overload.

Those excess iron as a result of repeated tranfusion couldn't possibly be excreted by our body, hence starting to accumulate in our body in the form of ferritin. When in excess, this will lead to a condition called as Haemosiderosis and Haemochromatosis.

Moreover, this excess iron tend to accumulate in the vital organs such as the heart (heart failure/cardiomyopathy), liver (hepatic cirrhosis), pancreas (diabetes mellitus) and endocrine organs (hypopituitarism and hypogonadism).

Therefore, iron chelation therapy is the choice of options in order for them not to get the side effects from repeated blood transfusion. Usually, iron chelation therapy is only needed after 20 transfusion have been done as each unit of blood tranfusion will give them an excess of 200-250mg of iron.

There are 3 form of iron chelating therapy available :

Desferrioxamine

- subcutaneous injection via an infusion pump (5-7x per week) for 8-12 hours
- indicated for those > 3 years old, ferritin > 1000 ug/l after 10-20 tranfusion
- promote urine excretion
- s.e = skin rashes, yersinia infection (fever, abdominal pain & diarrhea)
- cost - RM 500-1000

Deferiprone

- oral tablet form trice daily (tds)
- usually use in combination with desferrioxamine
- chelate iron from heart also
- s.e = agranulocytosis, joint pain,nausea, diarrhea & elevated liver enzymes

Desferasirox (Exjade)

- once daily dose tablet (OD)
- excrete iron via defaecation
- s.e = nausea/vomiting/abdominal pain, rashes,increased creatinine and liver enzymes


Other options available would include Splenectomy and Bone Marrow transplant

Spleenectomy

- when there is hypersplenism, large spleen or excess haemolysis
- indicated for those > 5 years old, transfusion of more than 200-220mls/kg/year.
- pancytopenia
- s.e = increased susceptibility to bacterial infection (meningococcus/ penumococcus/haemophilus influenza b) - need vaccination 4-6 weeks before surgery
thrombocytosis (use aspirin)

Bone Marrow Transplantation

- ideal choice of treatment (cure rate > 80%)
- allograph transplant from HLA-matched siblings
- s.e = infection, graft rejection and graft versus host disease
- greater chance of success rate if compliant to iron therapy, < 2cm of hepatomegaly and no portal fibrosis