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| Thalassaemia Patient in Bangladesh |
It is an inherited impairment of haemoglobin production, in which there is partial or complete failure to synthesis a specefic type of globin chain.
Types:
1. Beta thalassaemia
2. Alpha thalassaemia
Beta thalassaemia
On reduction of beta chain:
1. β0:
Complete absence of beta chain synthesis.
2. β+
:Incomplete
reduction of beta chain synthesis.
Clinical classification:
1. Thalassaemia major/cooley’s anemia:
Total suppression of beta chain
synthesis
2. Thalassaemia minor:
Suppression of beta chain synthesis is
less severe.
Clinical Triad of Thalassaemia
1. Anemia
2. Jaundice
3. Splenomegaly
Investigations
1) CBC
2) Haemoglobin
Electrophoresis(confirmatory)
3) Serum
iron profile
a) Serum
iron
b) Serum
ferritin
c) TIBC
4) x-ray
skull
a. Widdening
of diploic space
b. thinning
of outer table
Treatment:
1) Specific:
Haemopoietic stem cell transplantation (HSCT)
2) Supportive:
v Blood
transfusion (maintain more than 10mg/dl Hb)
v Folic
acid
v Iron
chelating agent: (Who need frequent blood transfusion)
i.
Oral: Deferiprone
ii.
Subcutaneous infusion: Desferrioxamine
3) Counseling
and screening
*Supertransfusion: Cronic blood
transfusion is given to patient to achieve haemoglobin concentration more than
11gm/dl.
*Hypertransfusion: Cronic blood
transfusion is given to patient to achieve haemoglobin concentration more than
14gm/dl.
REF:
Robi sir, Assistant professor of Medicine, MMCH