HYDROXYUREA THERAPY IN SICKLE-CELL-ANEMIA PATIENTS IN CURACAO, THE NETHERLANDS-ANTILLES

We have treated 9 patients with sickle cell anemia (SS) with hydroxyur ea (HU). All 9 patients carried 4 alpha-globin genes and the beta(s)-g lobin haplotypes 19/19 (Benin/Benin), except for 1 who had haplotype 1 9 together with type 3 (Benin/Senegal). Six patients received HU for 1 0 months and were again treated with the drug for 5 months after an in terval of 1 year. One patient was given HU for 22 consecutive months. A record was kept of hematological and biochemical data, Hb F and (G) gamma levels, as well as possible clinical complications. Our data sho w that HU generally improves the hematological and biochemical values and the level of Hb F, and reduces painful crises in some patients. Ho wever, although the clinical symptoms improved in some patients during HU therapy, the older patients did not observe any changes in their g eneral condition; the same is the case for the patient with haplotype 19/3. One patient also experienced life-threatening liver sequestratio n during treatment. We conclude that the selection of patients who may benefit from HU therapy needs further evaluation.