Abstract
More than 90% of children with sickle cell anemia (SCA) lose splenic function by the age of 2yrs. Splenic function may improve with hydroxyurea, but previous studies are conflicting. We prospectively evaluated the effect of hydroxyurea on splenic filtrative function. Children with SCA enrolled in the Hydroxyurea Study of Long-Term Effects (HUSTLE-NCT00305175) underwent clinical evaluations including Tc(99) m liver-spleen (LS) scans before hydroxyurea initiation and after 3yrs of treatment to maximum tolerated dose (MTD). LS scans were classified as follows: no uptake, <10% uptake, decreased but ≥10% uptake, and normal. Mean age (N=40) was 9.1yrs, range 2.3-17.0. After 3yrs of treatment, 13 (33%) had uptake on LS scan. These 13 children were younger (median age 6.0 vs. 10.6yrs, P=0.008), had a higher HbF at baseline (mean 10.2% vs. 5.8%, P=0.004) and after 3yrs (22.9% vs. 13.9%, P<0.001), achieved MTD more rapidly (median 288 vs. 358d, P=0.021), and were more likely to have baseline splenic uptake (P<0.001). Hydroxyurea at MTD is associated with preserved or improved splenic filtrative function, with 33% demonstrating LS scan uptake after 3yrs. Younger age, higher %HbF, and baseline splenic function are associated with a favorable outcome.
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