Optimal management of β thalassaemia intermedia

Ali T. Taher, Khaled M. Musallam, Maria Domenica Cappellini, David J. Weatherall

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish
Pages (from-to)512-523
Number of pages12
JournalBritish Journal of Haematology
Volume152
Issue number5
DOIs
Publication statusPublished - Mar 2011

Keywords

  • Fetal haemoglobin induction
  • Iron chelation
  • Splenectomy
  • Thalassaemia intermedia
  • Transfusion

ASJC Scopus subject areas

  • Hematology

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