Table 4Outcomes of Hydroxyurea Use in Sickle Cell Disease Reported by Genotype

Author, yearComments
Santos, 200259More marked improvement in splenic function in patients with Hb SS than in patients with HbSβ0-thalassemia. Thought to be due to less severely impaired splenic function at baseline in patients with Hb Sβ0-thalessemia
Loukopoulos, 200093No increase in Hb among patients with Hb Sβ+-thalassemia; increase in the Hb SS and HbSβ0-thalessemia groups. The β-thalassemia genotype did not affect Hb F response; substantial increase in all groups.
Zimmerman, 200481Hb F % increased from baseline in all genotypes except those with Hb SC disease. Patients with severe forms of SCD (Hb SS, HbS/β0, and Hb S/OArab) had significant increases in hemoglobin concentration, whereas patients with Hb SC or HbSβ+/ -thalassmia had minimal changes in Hb concentration. Patients with Hb SC tolerated less HU before toxicity developed.
el-Hazmi, 199245Increase in Hb F% for patients with Hb SS and patients with HbSβ0-thalessemia
Maier-Redelsperger, 199875All patients in study were homozygous SS; no difference in response to HU by β-globin gene haplotypes.

From: 3, Results

Cover of Hydroxyurea for the Treatment of Sickle Cell Disease
Hydroxyurea for the Treatment of Sickle Cell Disease.
Evidence Reports/Technology Assessments, No. 165.
Segal JB, Strouse JJ, Beach MC, et al.

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