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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Hemoglobin sickle-beta thalassemia


Other Names for this Disease
  • Hb S beta-thalassemia
  • Sickle beta thalassemia
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Symptoms


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What are the signs and symptoms of hemoglobin sickle-beta thalassemia?

Symptoms of hemoglobin sickle-beta thalassemia vary in severity depending on the beta thalassemia mutation inherited, which controls the amount of normal beta globin produced in the body. Beta globin is a component of hemoglobin, which is a protein in the red blood cells that carries oxygen.[1]

When no beta globin is produced by the HBB gene (called sickle cell-beta zero thalassemia), the condition is almost identical to sickle cell disease. These patients may have irreversibly sickled red blood cells, severe anemia, and frequent sickle cell crisis. When some beta globin is produced by the HBB gene (called sickle cell-beta plus thalassemia), the condition is less severe.[2]
Last updated: 4/14/2011

References
  1. Beta Thalassemia. Cooley's Anemia Foundation. http://www.cooleysanemia.org/updates/pdf/Beta_Thalassemia.pdf. Accessed 4/14/2011.
  2. Bender MA & Hobbs W. Sickle Cell Disease. GeneReviews. May 17, 2012; http://www.ncbi.nlm.nih.gov/books/NBK1377/. Accessed 12/20/2012.