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Previous Volume 340:1021-1030 April 1, 1999 Number 13 Next

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One of every 600 black people in the United States has sickle cell anemia. In addition, sickle cell–hemoglobin C disease and sickle cell–ß-thalassemia, which are other common genotypes of sickle cell disease, together are as common as sickle cell anemia. Sickle hemoglobin (hemoglobin S, ₂ ß₂^S) accounts for over half the hemoglobin in patients with these disorders. Eight percent of black Americans are heterozygous carriers of the sickle cell trait; about 40 percent of their hemoglobin is hemoglobin S. They do not have anemia and need neither treatment nor occupational restrictions. About 5 percent have hematuria at some time and . . . [Full Text of this Article]

Pathophysiology

Clinical Features

Principles of Treatment

General Measures

Treatment Directed at the Relief of Symptoms

Painful Episodes

Transfusion

Treatment Directed at the Prevention of Complications

Hydroxyurea

Transplantation

Experimental Therapy

Induction of Hemoglobin F by Short-Chain Fatty Acids

Membrane-Active Drugs

Other Experimental Treatments

Conclusions

References

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