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Volume 9 (2009), Pages 615-625
Review Article

Variable Clinical Phenotypes of α-Thalassemia Syndromes

Hematology/Oncology Department, Children's Hospital and Research Center (CHRCO), Oakland, CA, United States

Academic Editor: Susan Perrine

Copyright © 2009 Sylvia Titi Singer.


Genetic mutations of the α genes are common worldwide. In Asia and particularly Southeast Asia, they can result in clinically significant types of α-thalassemia, namely hemoglobin (Hb) H disease and Hb Bart's hydrops fetalis. The latter is generally a fatal intrauterine condition, while Hb H disease results in clinical complications that are frequently overlooked. The high prevalence of the carrier state and the burden of these diseases (and other α-thalassemia variants) call for more attention for improved screening methods and better care.