Hematologic, Immunologic, and Neoplastic Disorders Practice Test 2026 - Free Questions and Study Guide

Hydroxyurea is a disease-modifying therapy used in children with sickle cell disease. It works by increasing the production of fetal hemoglobin (HbF), which inhibits the polymerization of sickle hemoglobin (HbS) when oxygen levels drop. With more HbF, red cells are less prone to sickling, which translates into fewer painful vaso-occlusive crises, fewer episodes of acute chest syndrome, and often fewer hospitalizations and transfusion needs. This makes hydroxyurea a cornerstone of long-term management in pediatric patients who have frequent crises or severe disease manifestations. It’s not a cure and is not used to treat an acute crisis; rather, it is a ongoing therapy that requires regular monitoring, especially blood counts and organ function, to balance efficacy with potential bone marrow suppression. The other options don’t tackle the disease process: removing liver tissue isn’t a treatment for sickle cell disease, metabolic alkalosis therapy isn’t relevant to sickle cell pathophysiology, and while positive self-talk can aid coping, it doesn’t alter red blood cell sickling or disease progression.