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Thalassaemia is a disorder of haemoglobin
synthesis which is characterized by the absence or reduced synthesis of globin
chain synthesis of Hb (6). Thalassemia is prevalent in Malaysia.  The types of Thalassemia include
alpha-thalassemia, beta-thalassemia, haemoglobin (Hb) E, deltabeta-thalassemia
and Hb Constant Spring. It has been estimated that 1 in 4 person in Malaysia
carries one of the above genetic abnormalities. The ?
thalassaemias together with its heterozygous interaction with HbE disease
constitute the bulk of the patients’ load (7). It clinically presents as ?-thalassaemia trait (?0 or
?+), ?-thalassaemia intermedia (?+/?+; ?+/?0)
and ?-thalassaemia major (?0/?0).

 

The
most recent data from the Malaysian Thalassaemia Registry  in 2009 revealed that there are a total of
4,541 registered patients of thalassemia, 3,310 of which consist of the
transfusion dependent ? thalassaemia major and HbE ? thalassaemia patients.

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Meanwhile, the thalassaemia intermedia accounted for 455 patients.

?
thalassaemia major will present with anaemia, hepatosplenomegaly, jaundice,
thalassemia facies or a growth failure and growth retardation, earlier in life.

? thalassaemia intermediate presents slightly later in life with mild anaemia,
thalassemia facies and hepatosplenomegaly.

 

3.1.2
Management of Thalassemia in Malaysia

At presentation,
the patient will be monitored for the haemoglobin. Regular transfusion is
started when a patient is confirmed to have thalassaemia major and is unable to
maintain Hb > 7 g/dL or if there is poor growth (8). Transfusion
interval depends on pre- and post-transfusion Hb level and is usually 2 – 4
weeks apart.

 

 

Monthly
packed red blood cell (PRBC) transfusions will result in an iron intake of 0.3
– 0.5 mg/kg/day. The iron burden will be assessed based on the serum ferritin
level, cardiac iron concentration (MRI T2*), as well as the liver iron
concentration. After about 10 – 20 transfusions, most patients will have a
serum ferritin (SF) level exceeding 1000 ?g/L , an iron load considered high
enough to warrant iron chelation therapy. Thus, iron chelation therapy will be
initiated if the patient has received >10 units of blood and when the serum
ferritin exceeds 1000?g/L on more than two occasions at least two weeks apart (7).

 

For
an optimal iron chelation, the patient will be monitored for serum ferritin,
T2* of the heart and liver iron concentration (LIC). Adequate chelation is
regarded as serum ferritin <2,500?g/L, T2* of the heart >20ms and LIC
<7mg Fe/gm, the therapy is maintained. The aim is to reduce the serum ferritin until <1000 ?g/L.

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