Details

Autor(en) / Beteiligte

• Platt, Orah S

Titel

Hydroxyurea for the Treatment of Sickle Cell Anemia

Ist Teil von

• The New England journal of medicine, 2008-03, Vol.358 (13), p.1362-1369

Ort / Verlag

Boston, MA: Massachusetts Medical Society

Erscheinungsjahr

2008

Quelle

Free E-Journal (出版社公開部分のみ）

Beschreibungen/Notizen

• An 18-year-old woman with sickle cell anemia presents with recurrent painful crises, and treatment with hydroxyurea is recommended. Hydroxyurea causes a shift toward the production of red cells containing fetal hemoglobin. A possible increase in the risk of acute leukemia due to hydroxyurea therapy remains the subject of debate. An 18-year-old woman with sickle cell anemia presents with recurrent painful crises, and treatment with hydroxyurea is recommended. Hydroxyurea causes a shift toward the production of red cells containing fetal hemoglobin. Foreword This Journal feature begins with a case vignette that includes a therapeutic recommendation. A discussion of the clinical problem and the mechanism of benefit of this form of therapy follows. Major clinical studies, the clinical use of this therapy, and potential adverse effects are reviewed. Relevant formal guidelines, if they exist, are presented. The article ends with the author's clinical recommendations. An 18-year-old woman with sickle cell anemia has had increasing symptoms, with painful crises and episodes of the acute chest syndrome. She was hospitalized three times in the past year. A hematologist recommends that hydroxyurea therapy be started. The Clinical Problem There are about 50,000 people in the United States who are homozygous for the sickle hemoglobin gene and thus have sickle cell anemia. Sickle cell anemia is primarily seen in persons of African heritage, about 1 in 14 of whom is an asymptomatic carrier (a heterozygote). One in 700 newborns of African heritage is affected. 1 Although it is the most severe of the common sickle cell diseases (which include hemoglobin SC disease and sickle β 0 -thalassemia), patients with sickle cell anemia have a wide spectrum of clinical manifestations. All patients with this disorder have a chronic hemolytic . . .