Thalassemia refers to a group of hereditary diseases caused by a defect in alpha or beta globin synthesis [1]. This impaired synthesis leads to a reduced supply of globin chains and results in the malformation of hemoglobin, which gives rise to microcytic hypochromic anemia. However, since synthesis of the unaffected globin occurs at a normal rate, the alpha or beta subunits accumulate dispropo...

Most α-thalassemia cases are caused by deletions of the structural α-globin genes. The degree of microcytosis and hypochromia has been correlated with the number of affected α-globin genes, suggesting a promising role of hematologic parameters as predictive diagnostic tools. However, cut-off points for these parameters to discriminate between the different subtypes of α-thalassemia are yet to b...

The relevant investigations and management of a case of alpha-thalassaemia major suspected antenatally is discussed. The value of ultrasonically guided cordocentesis in the definite diagnosis of this condition is emphasised in the management of this pregnancy. We believe that this is the first time such a procedure has been done in this country.

PURPOSE To study the validity and reliability of the Malay version of the Specific Thalassemia Quality of Life Instrument (STQOLI) in Sabah's adult thalassemia patients. PATIENTS AND METHODS This cross-sectional study was done at Thalassemia Treatment Centre, Queen Elizabeth Hospital in Sabah, Malaysia. Eighty-two adult thalassemia patients who fulfilled the inclusion and exclusion criteria w...