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International Journal of Hematology
Published in: International Journal of Hematology 1/2018

01-07-2018 | Review Article

β-Thalassemia intermedia: a comprehensive overview and novel approaches

Authors: Chingiz Asadov, Zohra Alimirzoeva, Tahira Mammadova, Gunay Aliyeva, Shahla Gafarova, Jeyhun Mammadov

Published in: International Journal of Hematology | Issue 1/2018

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Abstract

β-Thalassemia intermedia is a clinical condition of intermediate gravity between β-thalassemia minor, the asymptomatic carrier, and β-thalassemia major, the transfusion-dependent severe anemia. It is characterized by a significant clinical polymorphism, which is attributable to its genetic heterogeneity. Ineffective erythropoiesis, chronic anemia, and iron overload contribute to the clinical complications of thalassemia intermedia through stepwise pathophysiological mechanisms. These complications, including splenomegaly, extramedullary erythropoiesis, iron accumulation, leg ulcers, thrombophilia, and bone abnormalities can be managed via fetal hemoglobin induction, occasional transfusions, chelation, and in some cases, stem cell transplantation. Given its clinical diversity, thalassemia intermedia patients require tailored approaches to therapy. Here we present an overview and novel approaches to the genetic basis, pathophysiological mechanisms, clinical complications, and optimal management of thalassemia intermedia.
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Metadata
Title
β-Thalassemia intermedia: a comprehensive overview and novel approaches
Authors
Chingiz Asadov
Zohra Alimirzoeva
Tahira Mammadova
Gunay Aliyeva
Shahla Gafarova
Jeyhun Mammadov
Publication date
01-07-2018
Publisher
Springer Japan
Published in
International Journal of Hematology / Issue 1/2018
Print ISSN: 0925-5710
Electronic ISSN: 1865-3774
DOI
https://doi.org/10.1007/s12185-018-2411-9

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