Do you recommend hydroxyurea in patients with sickle cell disease with hereditary persistence of fetal hemoglobin who have recurrent vaso-occlusive crises requiring hospital admission?

It’s complicated. Patients with sickle cell-HPFH that is due to deletion of the beta-globin gene usually have 30% HbF spread nearly evenly among their red cells (pancellular), and as a result, have almost normal hematology and rarely have sickle vasoocclusive events. They do not require treatment. (...