A brief account of the investigations and management of beta thalassemia major

1. Investigations and
Management of
Thalassemias
Lavina Belayutham

2. Investigations
1. Peripheral smear

3. 2. Hemoglobin electrophoresis
- Markedly reduced levels of HbA
- Raised levels of HbF
3. Osmotic fragility test
- Increased resistance to hemolysis

4. 4. Skull radiograph – “hair on end”appearance

5. Treatment of beta-thalassemia major
1. Regular transfusions every 4-6 weeks
(maintain Hb>10gm/dL)
2. Folic acid supplementation (5mg daily)
3. Splenectomy
4. Allogenic bone marrow transplantation from
HLA-compatible sibling

6. Treatment of iron overload
1. Desferrioxamine (overnight subcutaneous
infusion on 5-7 nights each week)
2. Ascorbic acid 200mg daily
3. Other iron chelator : deferasirox, deferiprone

7. Treatment of iron overload
1. Desferrioxamine (overnight subcutaneous
infusion on 5-7 nights each week)
2. Ascorbic acid 200mg daily
3. Other iron chelator : deferasirox, deferiprone