Hb H disease resulting from the association of an αº-thalassemia allele [-(α)20.5] with an unstable α-globin variant [Hb Icaria]: first report on the occurrence in Brazil

Hb H disease resulting from the association of an αº-thalassemia allele [-(α)20.5] with an unstable α-globin variant [Hb Icaria]: first report on the occurrence in Brazil

Hb H Disease is caused by the loss or inactivation of three of the four functional a-globin genes. Patients present chronic hemolytic anemia and splenomegaly. In some cases, occasional blood transfusions are required. Deletions are the main cause of this type of thalassemia (α-thalassemia). We descr...

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Bibliographic Details
Main Authors: Elza M. Kimura, Denise M. Oliveira, Kleber Fertrin, Valéria R. Pinheiro, Susan E.D.C. Jorge, Fernando F. Costa, Maria de Fátima Sonati
Format: Article
Language:English
Published: Sociedade Brasileira de Genética 2009-01-01
Series:Genetics and Molecular Biology
Subjects:
hereditary hemoglobinopathies
alpha-thalassemia
Hb H disease
Hb Icaria
Online Access:http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1415-47572009000400007&lng=en&tlng=en

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http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1415-47572009000400007&lng=en&tlng=en

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