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Clinical Pediatrics
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Sickle Cell Anemia: Targeting the Role of Fetal Hemoglobin in Therapy

Emma Coleman, BSc

St Thomas Hospital, London, United Kingdom

Baba Inusa, MBBS, FRCPCH, DCP (Haem), FMCPaed, MRCP

St Thomas Hospital, London, United Kingdom, Baba.Inusa{at}gstt.nhs.uk

Sickle cell anemia results from the single amino acid substitution of valine for glutamic acid in the ß-chain owing to a nucleotide defect that causes the production of abnormal ß-chains in hemoglobin S. Abnormal hemoglobin chains form polymers in the deoxygenated state, leading to the characteristic sickle cells. The polymerization of deoxygenated hemoglobin S accounts for the pathologic changes in sickle cell disease. The main-stay of therapy in sickle cell disease aims to reduce the amount of sickled hemoglobin present through the prevention of polymerization and reversal of this process. One way of discouraging polymerization is to increase the level of fetal hemoglobin, which because of its high oxygen affinity, does not participate in the polymerization process. Fetal hemoglobin production may be induced pharmacologically or by the use of gene therapy and genetic engineering techniques.

Key Words: sickle cell anemia • fetal hemoglobin • hydroxyurea therapy • anemia • painful crisis

Clinical Pediatrics, Vol. 46, No. 5, 386-391 (2007)
DOI: 10.1177/0009922806297751
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