Contemporary approaches to treatment of beta-thalassemia intermedia.
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.
American University of Beirut Medical Center, Beirut, Lebanon. ataher@aub.edu.lb
Ali T
AT
Taher
Khaled M
KM
Musallam
Mehran
M
Karimi
Maria D
MD
Cappellini
D001803:Blood Transfusion; D006801:Humans; D007502:Iron Chelating Agents / Q000627:therapeutic use; D013156:Splenectomy; D017086:beta-Thalassemia / Q000188:drug therapy / Q000601:surgery / Q000628:therapy*
D007502:Iron Chelating Agents