Treatment of sickle cell anemia with hydroxyurea and erythropoietin

Article Abstract:

Sickle cell disease is a hereditary condition in which the red blood cells are abnormally shaped and resemble a crescent, or sickle. These cells are crescent-shaped because they contain hemoglobin S, an abnormal form of hemoglobin. The sickled cells block blood flow through the capillaries (tiny blood vessels), reducing oxygen delivery to tissues and causing fever and severe pain. In this study, two treatments for sickle cell disease were tested in five patients. The treatments were hydroxyurea and erythropoietin; hydroxyurea is known to stimulate production of hemoglobin F, and erythropoietin may also increase this form of hemoglobin. Hemoglobin F is the hemoglobin contained in the red blood cells of the fetus, and it has been suggested that increased levels might help to alleviate symptoms in sickle cell patients. All five subjects were given increasing intravenous doses of erythropoietin for eight weeks. Approximately six weeks after the erythropoietin was discontinued, three of the five subjects were then given hydroxyurea orally until the proper dose was determined. In the last phase of the study, hydroxyurea was continued and erythropoietin was resumed for a period of four weeks. It was found that erythropoietin had no beneficial effects, either when used alone or with hydroxyurea. However, hydroxyurea did increase hemoglobin F production and reduced two manifestations of sickle cell disease: the rate of hemolysis (destruction of red blood cells) and the polymerization (linking into larger molecules) of hemoglobin S within the cell. The three patients given hydroxyurea for about one year reported almost no pain crises, which is promising, but the number of subjects was small and the improvements could have been coincidental or related to the extra medical attention the patients received. Large, controlled studies of hydroxyurea in sickle cell patients are needed. (Consumer Summary produced by Reliance Medical Information, Inc.)

Drug therapy, Erythropoietin

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Sickle cell anemia - basic research reaches the clinic

Article Abstract:

A drug that may prevent sickle cell crises illustrates how basic medical research can lead to effective treatments. Sickle cell anemia is caused by a type of hemoglobin called hemoglobin S, which causes blood cells to sickle. In 1948, a pediatrician noted that newborn infants at risk for sickle cell anemia were temporarily protected by high levels of fetal hemoglobin, or hemoglobin F. Hemoglobin F prevents the sickling of red blood cells that causes sickle cell crises. Hydroxyurea is a drug that can increase the production of hemoglobin F in red blood cells. A 1995 study found that hydroxyurea can reduce the number of sickle cell crises in adults with sickle cell anemia. However, the drug has severe side effects and its effects in growing children are unknown. Researchers may find other ways of increasing hemoglobin F, including gene therapy. Bone marrow transplants may also be effective in sickle cell anemia and other disorders of hemoglobin synthesis.

Editorial

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