dear mba Fitri,
aku ada infonya, tapi in english...
translete sendiri ya ;-)
Synonyms
Cooley's anaemia
Leptocytosis
Mediterranean anaemia
Description
Thalassaemia is one of the most common inherited disorders and is
particularly prevalent in individuals of Mediterranean, Southeast
Asian and African origin. It is characterised by defective
haemoglobin synthesis (the haemoglobin molecules are normal but
their number is decreased - the affected Hb chain may be
virtually undetectable in very severe disease). As a result,
haemoglobin synthesis is reduced and anaemia results.
The normal haemoglobin molecule has 4 polypeptide chains; 2 alpha
and 2 beta. In thalassaemia, one or more of the chains is
reduced. Depending on which chain is involved, the sufferer has
alpha or beta thalassaemia.
Disease classification
Alpha-thalassaemia
In contrast to beta thalassaemia, alpha-thalassaemia is
caused by gene deletions of one or more of the four
alpha-chain genes.
There are various levels of severity, depending upon the
number of alpha-chain genes deleted:
One or two alpha-chain genes deleted -
alpha-thalassaemia traits. Anaemia is, at most, mild
or absent. There is microcytosis.
Three alpha-chain genes deleted (HbH disease)-
variable severity of anaemia from mild to moderate.
Splenomegaly may be present. Not usually transfusion
dependent.
Four alpha-chain genes deleted - no alpha-chain
synthesis. Incompatible with life.
Beta-thalassaemia
Each individual inherits two beta-chain genes from his/her
parents (one from each). Clinically, there are three forms
of the disorder:
Beta-thalassaemia minor (trait) - the common, symptomless,
heterozygous carrier state resulting from inheriting the
gene from only one parent (who also has the trait). Anaemia
is mild or absent (Hb no lower than 10 mg/dl). Thalassaemia
minor is suspected when there is a known family history of
thalassaemia and the blood profile shows a mild anaemia,
low MCV (microcytosis) and an increased red blood cell
count.
Beta-thalassaemia intermedia - characterised by moderate
anaemia (Hb 7-10g/dl). The genetic abnormalities underlying
this form of the disease are complex. Transfusion is rarely
necessary and is avoided if possible because of the risk of
iron overload.
Beta-thalassaemia major (Cooley's anaemia) - the homozygous
form resulting from the inheritance of an abnormal beta
chain gene from both parents. It is characterised by severe
anaemia (typically Hb is <6.0 g/dl), requiring regular
transfusion.
Prognosis
Alpha-thalassaemia trait features, at most, only mild anaemia and
life expectancy is normal.
HbH disease varies in severity. The prevalence appears to be
increasing as a result of the screening of populations at risk
and in these circumstances the anaemia may be mild and the
prognosis reasonable. However, a moderately severe haemolytic
anaemia with or without splenectomy can occur.
Beta-thalassaemia minor is one of the most common
haemoglobinopathies and accounts for virtually all the cases of
thalassaemia seen in life insurance. No treatment is necessary
and life expectancy is normal.
Beta-thalassaemia intermedia has a variable life expectancy.
Beta thalassaemia major (Cooley's anaemia) has a poor prognosis.
Treatment consists of transfusion and in some cases splenectomy.
Many patients do not survive beyond puberty and morbidity is
common. Thalassaemia major is rarely seen in life insurance as
life expectancy is short and it is a disease of chilhood.
Thanks n Regards
Corry. H
PT Prudential Life Assurance
Operational Support
Menara Thamrin ? Prudential Centre
Jl. MH. Thamrin Kav.3
Jakarta 10250
Ph. 62-21-2355.8888
Fitri-Yahoo
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[Ayahbunda-Online] (urgent)
01/09/2006 02:33 thalassemia major
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Please respond to
Ayahbunda-Online@
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dear pembaca milis tercinta...
ada yang tau tentang thalassemia major gak?
tolong dong infonya? ada para dokter kah yg berkumpul di ayahbunda milis
ini?
apakah TM itu sejenis kanker darah?
Salam
Fitri
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