Dose Adjustment/ Noncompliance in a Thalassaemia Patient. Background. Adequate dose titration and compliance with chelation therapy are crucial factors in achieving prolonged patient survival
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Dose Adjustment/Noncompliance in a Thalassaemia Patient
1. Brittenham GM et al. N Engl J Med. 1994;331:567-573.
Iron Overloaded State
LIC (mg Fe/g dw)
Serum ferritin (ng/mL)
>1000 to <2500
Transferrin saturation (%)
Alanine aminotransferase (U/L)
Labile plasma iron (μM)
Increased risk of complications
Increased risk of cardiac disease
Courtesy of A. Taher, MD.
What should the next step be?
A. Counsel patient about the importance of compliance and continue him on desferrioxamine
B. Increase desferrioxamine dosage
C. Switch to oral deferasirox
aAlthough 20 mg/kg/d is the usual starting dose of deferasirox, 10 mg/kg/d or 30 mg/kg/d can also be used, depending on transfusion frequency1.
1 European Agency for Evaluation of Medicinal Products guidelines, 2007.
Recommended initial deferasirox dose
pRBCs >14 mL/kg/mo(~4 adult units)
pRBCs <7 mL/kg/mo(~2 adult units)
Maintenance of body iron
Desferrioxamine 40 mg/kg/d for 5 days per week
Deferasirox 20 mg/kg/d
Reduction of body iron
Starting doses may also be modified as follows:
For patients well managed on desferrioxamine, suggested starting dose may be numerically half desferrioxamine dose, eg:
EXJADE® (deferasirox) Basic Prescribing Information. Novartis Pharma AG. National Prescribing Information should be followed.
Approximately 2 weeks after starting deferasirox, the patient developed skin rash of moderate severity. How should the rash be managed?
A. Continue treatment
B. Stop treatment; reintroduce drug at a low dose after rash has resolved
C. Stop treatment; reintroduce drug at low dose in combination with steroid after rash has resolved
Continue treatment without interruption
Mild to moderate rash
More severe rash
Deferasirox Basic Prescribing Information. Novartis Pharma AG. National Prescribing Information should be followed.
Deferasirox increased to
Courtesy of A. Taher, MD
What might account for the increase in serum ferritin noted after month 9?
B. Increased transfusion requirement
C. Noncompliance with treatment
Liver Iron Concentration
Courtesy of A. Taher, MD.