International Journal of Hematology

, Volume 108, Issue 1, pp 5–21 | Cite as

β-Thalassemia intermedia: a comprehensive overview and novel approaches

  • Chingiz Asadov
  • Zohra Alimirzoeva
  • Tahira Mammadova
  • Gunay Aliyeva
  • Shahla Gafarova
  • Jeyhun Mammadov
Review Article


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.


β-Thalassemia intermedia Non-transfusion-dependent thalassemia Transfusion Complications Novel treatment 


Compliance with ethical standards

Conflict of interest

The authors declare no conflict of interest.


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Copyright information

© The Japanese Society of Hematology 2018

Authors and Affiliations

  • Chingiz Asadov
    • 1
  • Zohra Alimirzoeva
    • 1
  • Tahira Mammadova
    • 1
  • Gunay Aliyeva
    • 1
  • Shahla Gafarova
    • 1
  • Jeyhun Mammadov
    • 2
  1. 1.Institute of Hematology and TransfusiologyBakuAzerbaijan
  2. 2.Thalassemia CentreBakuAzerbaijan

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