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When To Begin Transfusion Therapy

Transfusion therapy should only begin once a diagnosis of thalassaemia major is confirmed. A confirmed diagnosis of Thalassaemia major is based on:

•  laboratory test
•  genetic analysis

The severity of anaemia is usually assessed on the basis of levels of haemoglobin (Hb) in the blood. This is measured in grams (g) per decilitre (dI - 1/100 th of a litre) of blood. A normal Hb level is generally considered to be between 13-16g/dI in men and 11/14g/dI in woman and children. In both men and women, haemoglobin levels of between 8-11g/dI represent moderate anaemia, with severe anaemia at levels less than 8g/dI.

Patients should only begin transfusion therapy once thalassaemia has been confirmed and when:

* Hb levels are registered at less than 7g/dl on two successive occasions. Paients may thrive and grow normally with Hb values between 6-7g/dl but a decision not to transfuse under these circumstances requires great clinical experience and careful observation.

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Important Note

* Hb levels are >7g/dl but accompanying physical characteristics are noted, such as:

-facial changes
-poor growth and limited weight gain
-bone fractures
-extra-medullar hematopoiesis, resulting in tumor masses.

Where these criteria are observed, transfusion therapy should not be delayed.

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