Journal
BRITISH JOURNAL OF HAEMATOLOGY
Volume 152, Issue 5, Pages 512-523Publisher
WILEY
DOI: 10.1111/j.1365-2141.2010.08486.x
Keywords
thalassaemia intermedia; splenectomy; transfusion; iron chelation; fetal haemoglobin induction
Categories
Ask authors/readers for more resources
P>Our understanding of the molecular and pathophysiological mechanisms underlying the disease process in patients with beta thalassaemia intermedia (TI) has substantially increased over the past decade. The hallmark of disease process in patients with TI includes ineffective erythropoiesis, chronic haemolytic anaemia, and iron overload. There are a number of options currently available for managing patients with TI including splenectomy, transfusion therapy, iron chelation therapy, modulation of fetal haemoglobin production, and several other agents targeting specific clinical complications. Limited studies assessed the efficacy and safety of these modalities; hence, there are currently no clear guidelines for managing patients with TI. Until solid evidence-based guidelines are available, individualised treatment should be entertained.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available