Thalassemia: The Daily PANCE Blueprint
A 3-year-old girl with beta-thalassemia major is brought to the clinic for follow-up. She receives monthly packed red blood cell transfusions. The serum ferritin level is 1200 ng/mL. Which of the following is the most appropriate next step in management?
A) Increase transfusion frequency
B) Start iron chelation therapy
C) Order MRI for iron overload monitoring
D) Splenectomy
E) Allogeneic hematopoietic stem cell transplant
Answer and topic summary
The answer is B) Start iron chelation therapy
This young patient with beta-thalassemia major requires chronic red blood cell transfusions, putting her at risk for iron overload. Iron overload is common because of increased absorption (due to defective erythropoiesis) and frequent transfusions. Iron chelation therapy is generally initiated when the serum ferritin level is >1000 ng/mL after about 1-2 years of regular transfusions to remove excess iron and prevent complications. Splenectomy may help decrease transfusion requirements for patients with significant splenomegaly.
- A) Increasing transfusion frequency could worsen iron overload.
- C) MRI can be used to monitor iron deposition, but chelation should be started based on the ferritin level.
- D) Splenectomy may help reduce transfusion needs but does not address iron overload.
- E) Transplantation is curative, but iron overload should still be addressed first.
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