Prognosis
Beta-thalassaemia trait
Normal life expectancy.
Beta-thalassaemia intermedia
May have significant cosmetic changes in appearance, which may interfere with quality of life. Will have iron overload to a variable degree, and morbidity may be dependent on the management of this complication. Co-inheritance of other mutations, such as those for hereditary haemochromatosis, Gilbert's syndrome, or thrombophilic syndromes, may modify the morbidity of this condition.[61]
Complications related to transfusional iron overload, which develop in those patients who receive multiple transfusions, are similar to those with beta-thalassaemia major.
Beta-thalassaemia major
If untreated, beta-thalassaemia major is usually fatal in the first few years of life, death being the result of heart failure secondary to severe anaemia. Regular transfusions allow for a markedly improved quality of life and survival, the latter varying based on how recently the person was born. With improvements in transfusion and chelation practices, survival has improved dramatically over the past 3 to 4 decades, with people born in the past 20 years or so expected to have near-normal survival if treated appropriately.[74]
The leading cause of death remains heart failure, which results from severe iron-induced cardiomyopathy in poorly chelated patients. Other complications (e.g., infection risk/splenectomy complications) cause significant morbidity.[75]
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