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Nursing QUESTION #9267
Question 1
A 5-year-old child with thalassemia major requires a blood transfusion every 3–4 weeks. After 5 years of regular transfusions, the child develops bronze skin discoloration, hepatomegaly, growth retardation, and cardiac arrhythmias. The PRIMARY cause of these complications and its MANAGEMENT is:
  • Repeated infections from transfusions; prophylactic antibiotics
  • Iron overload (hemosiderosis); iron chelation therapy with desferrioxamine or deferasirox✔️
  • Folate deficiency; folic acid supplementation
  • Autoimmune hemolysis; immunosuppressive therapy
Correct Answer Explanation

Beta-Thalassemia Major (Cooley's Anemia) — requires chronic blood transfusions leading to Iron Overload (Hemosiderosis/Hemochromatosis).

Mechanism:

\[\text{Each unit pRBC} \approx 200{-}250\,\text{mg iron}\]

Human body has no active iron excretion mechanism → iron accumulates in organs:

  • Liver: Hepatomegaly, cirrhosis
  • Heart: Cardiomyopathy, arrhythmias (leading cause of death in thalassemia)
  • Endocrine glands: Diabetes mellitus, hypothyroidism, hypogonadism, growth retardation
  • Skin: Bronze discoloration

Monitoring: Serum ferritin (target \(<1000\,\mu\text{g/L}\)), MRI T2* (cardiac iron)

Iron Chelation Therapy:

DrugRouteDoseSide Effects
Desferrioxamine (DFO)SC (8–12 hr infusion) or IV\(25{-}50\,\text{mg/kg/day}\)Auditory/visual toxicity
Deferasirox (Exjade)Oral\(20{-}40\,\text{mg/kg/day}\)GI upset, renal toxicity
DeferiproneOral\(75\,\text{mg/kg/day}\)Agranulocytosis

Curative treatment: Bone marrow/hematopoietic stem cell transplantation.