NCLEX Examination.
Practice Question # 732.
NCLEX Examination.
Thalassemia Major:
Thalassemia major (Cooley’s anemia) is characterized by severe anemia, marked hemolysis, and ineffective erythropoiesis (production of RBCs). With early regular transfusion therapy, growth and development through childhood are facilitated. Organ dysfunction due to iron overload results from the excessive amounts of iron obtained through the RBC transfusions. Regular chelation therapy (eg, via subcutaneous deferoxamine) has reduced the complications of iron overload and prolonged the life of these patients. This disease is potentially curable by BMT if the procedure can be performed before damage to the liver occurs (ie, during childhood).