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Optimal management of β thalassaemia intermedia
Author(s) -
Taher Ali T.,
Musallam Khaled M.,
Cappellini Maria Domenica,
Weatherall David J.
Publication year - 2011
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.2010.08486.x
Subject(s) - medicine , erythropoiesis , intensive care medicine , ineffective erythropoiesis , splenectomy , disease , thalassemia , transfusion therapy , anemia , chelation therapy , etiology , modalities , blood transfusion , spleen , social science , sociology
Summary Our understanding of the molecular and pathophysiological mechanisms underlying the disease process in patients with β 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.