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Thalassemia intermedia today: should patients regularly receive transfusions?
Author(s) -
Aessopos Athanassios,
Kati Maria,
Meletis John
Publication year - 2007
Publication title -
transfusion
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.045
H-Index - 132
eISSN - 1537-2995
pISSN - 0041-1132
DOI - 10.1111/j.1537-2995.2007.01192.x
Subject(s) - medicine , thalassemia , anemia , beta thalassemia , disease , hemolytic anemia , blood transfusion , extramedullary hematopoiesis , ineffective erythropoiesis , transfusion therapy , hemoglobinopathy , surgery , gastroenterology , pediatrics , haematopoiesis , erythropoiesis , stem cell , biology , genetics
BACKGROUND: β‐Thalassemia is an inherited hemoglobin disorder characterized by reduced synthesis of β‐globin chain. The severity of clinical course distinguishes this heterogeneous disease in two main subtypes: thalassemia major (TM) and thalassemia intermedia (TI). TI has a later clinical onset with a milder anemia that does not require transfusions at least during the first few years of life. The clinical picture of TI patients who have not received transfusions or have occasionally received transfusions is dominated by the consequences of chronic hemolytic anemia, tissue hypoxia, and their compensatory reactions, such as bone deformities and fractures, extramedullary hemopoiesis, spleen and liver enlargement, hypercoagulability, and pulmonary hypertension. These complications, especially the latter two, are getting more frequent and severe over the years. Nowadays, although TI patients have almost no changes in the course of the disease, well‐treated TM patients with regular transfusion‐chelation therapy showed suppression of the anemia‐related disorders in parallel to prolongation of life. The new oral iron chelators and the magnetic resonance imaging application for early detection of heart iron load are promising for further improvement on survival. CONCLUSIONS: Considering the current cost‐benefit balance of regular treatment in TM as well as the frequency and severity of complications in TI, it seems that the majority of TI patients will be benefited if this kind of treatment is applied targeting prevention and not palliation of the anemia‐induced complications.