alexa The role of ineffective erythropoiesis in non-transfusion-dependent thalassemia.
Haematology

Haematology

Journal of Blood Disorders & Transfusion

Author(s): Rivella S

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Abstract Ineffective erythropoiesis is the hallmark of beta-thalassemia that triggers a cascade of compensatory mechanisms resulting in clinical sequelae such as erythroid marrow expansion, extramedullary hematopoiesis, splenomegaly, and increased gastrointestinal iron absorption. Recent studies have begun to shed light on the complex molecular mechanisms underlying ineffective erythropoiesis and the associated compensatory pathways; this new understanding may lead to the development of novel therapies. Increased or excessive activation of the Jak2/STAT5 pathway promotes unnecessary disproportionate proliferation of erythroid progenitors, while other factors suppress serum hepcidin levels leading to dysregulation of iron metabolism. Preclinical studies suggest that Jak inhibitors, hepcidin agonists, and exogenous transferrin may help to restore normal erythropoiesis and iron metabolism and reduce splenomegaly; however, further research is needed. Copyright © 2012 Elsevier Ltd. All rights reserved.
This article was published in Blood Rev and referenced in Journal of Blood Disorders & Transfusion

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