MANAGEMENT OF THALASSAEMIA

Authors :Dr Wong Swee Lan , Dr Ng Hoong Phak , Dr Tam Pui Ying , Dr Hung Liang Choo
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Thalassaemias and Sickle cell disorder, are the commonest human inherited haemoglobin disorders that can be treated effectively, and also prevented at community level.
Thalassaemia is the commonest single gene disorder in Malaysia and a paradigm of monogenetic diseases. In 1995, it was estimated that about 8,000 persons were afflicted with HbE beta Thalassaemia, and 8,000 with Homozygous beta-Thalassaemia, about 40% of whom were dependent on regular blood transfusions for survival (Kaur, 1995). In addition, the carrier rate for beta Thalassaemia and HbE is estimated to be 3-5%.
Besides blood transfusion, another curative option is the use of haemopoietic stem cell transplantation. In Malaysia, it is currently being offered to potential patients with suitable donors at two centres, namely, University Malaya Medical Centre and the Paediatrics Institute, Kuala Lumpur Hospital.

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