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Blood, 1 October 2005, Vol. 106, No. 7, pp. 2269-2275.
Prepublished online as a Blood First Edition Paper on June 14, 2005; DOI 10.1182/blood-2004-12-4973.
 Previous Article | Next Article 
Submitted January 3, 2005
Accepted May 25, 2005
Long-term hydroxyurea therapy for infants with sickle cell anemia - the Husoft extension study
Jane S Hankins*, Russell E Ware, Zora R Rogers, Lynn W Wynn, Peter A Lane, J P Scott, and Winfred C Wang
St. Jude Children's Research Hospital, Memphis, TN, USA
University of Texas Southwestern Medical Center, Dallas, TX, USA
Emory University School of Medicine, Atlanta, GA, USA
Medical College of Wisconsin, Milwaukee, WI, USA
* Corresponding author; email: jane.hankins{at}stjude.org.
The long-term efficacy and toxicity of hydroxyurea for infants are undefined and its role in preventing organ dysfunction is unknown. Short-term feasibility of hydroxyurea administration, toxicities, hematologic effects, and effect on spleen function in infants with sickle cell anemia (SCA) was reported (HUSOFT trial). These infants completing two years of hydroxyurea therapy (20 mg/kg/day) were offered study extension with dose escalation to 30 mg/kg/day. Patients were monitored with laboratory tests and biannual imaging studies. Hematologic indices were compared with predicted age-specific values and event rates compared with historical rates. All twenty-one subjects completing the original trial enrolled in the extension study: median age 3.4 years-old (range 2.6-4.4); 12 females; 20 with Hb SS, one with Hb S/ °-thalassemia. Seventeen patients completed 4 years of hydroxyurea, and 11 completed 6 years. After four years, hydroxyurea was associated with increased hemoglobin concentration, %HbF, and MCV, and decreased reticulocytes, WBC, and platelets (p< 0.01). Patients experienced 7.5 acute chest syndrome (ACS) events/100 person-years, compared with 24.5 events/100 person-years among historical controls (p=0.001). Treated patients had better spleen function than expected and improved growth rates. Infants with SCA tolerate prolonged hydroxyurea therapy with sustained hematologic benefits, fewer ACS events, improved growth, and possibly preserved organ function.
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