Contemporary approaches to treatment of beta-thalassemia intermedia

Ali T. Taher, Khaled M. Musallam, Mehran Karimi, Maria D. Cappellini

Research output: Contribution to journalArticlepeer-review

Abstract

Beta-thalassemia intermedia (TI) is associated with a variety of serious clinical complications that require proactive and comprehensive management. These include skeletal deformities and osteopenia, compensatory extramedullary hematopoiesis and tumor formation, progressive splenomegaly, a hypercoagulable state resulting in thromboembolic events and pulmonary hypertension, and increased gastrointestinal iron absorption that often results in nontransfusional iron overload and liver damage. Although TI is generally considered a non-transfusion-dependent thalassemia, transfusion therapy may be an important part of the comprehensive management of this disease. This review describes the current state of the art for medical management of TI, with particular focus on the roles of splenectomy, transfusion, and iron chelation therapy.

Original languageEnglish
JournalBlood Reviews
Volume26
Issue numberSUPPL.1
DOIs
Publication statusPublished - Apr 1 2012

Keywords

  • Beta-thalassemia intermedia
  • HbF induction
  • Iron chelation
  • Splenectomy
  • Transfusion
  • Treatment

ASJC Scopus subject areas

  • Hematology
  • Oncology

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