EXPLANATION
The correct answer is hydroxyurea. Sickle cell disease is caused by a mutation of the β Hb chain. This causes polymerization of HbS and its characteristic sickle shape. These abnormal RBCs occlude capillary beds and lead to the many clinical manifestations of SCA. These manifestations include painful vaso-occlusive crises that include the acute chest syndrome. Treatment of acute crises is mainly supportive and includes oxygen and narcotic analgesics.
The rate of sickling in patients with sickle cell anemia is influenced by the intracellular concentration of hemoglobin S and by the presence of other hemoglobins within the cell. Hemoglobin F cannot participate in polymer formation, and its presence markedly retards sickling. Hydroxyurea increases the production of hemoglobin F and is therefore helpful in improving the quality of life in patients with frequent painful crises.
Folic acid supplementation and iron supplementation are used to treat anemia caused by either folate or iron. Although these may be helpful in treating anemia in patients with sickle cell anemia, they do not alter the frequency of painful crises.
Azathioprine is used in the treatment of autoimmune hemolytic anemia, but it has no effect on the course of sickle cell anemia.
Prednisone is also used in the treatment of autoimmune causes of hemolytic anemia but has no role in the management of patients with sickle cell anemia.
REFERENCES
- Primack BA, Mahaniah KJ. Anemia. In: South-Paul JE, Matheny SC, Lewis EL, eds. CURRENT Diagnosis & Treatment: Family Medicine. 4th ed. New York: McGraw-Hill; 2017.
- Damon LE, Andreadis C. Blood Disorders. In: Papadakis MA, McPhee SJ, Rabow MW, eds. Current Medical Diagnosis & Treatment 2017. New York: McGraw-Hill; 2017.
