Chelation strategies in adult patients with β-TM
| . | DFO* . | DFP . | DFO + DFP combination . | DFX . |
|---|---|---|---|---|
| T2* ≥ 20 ms | ||||
| Iron intake < 0.3 mg/kg/d | ||||
| LIC ≥ 15 mg Fe/g dw | 40-50 mg/kg per day, 8-10 h/d, 6 or 7 d/wk, SQ | 75-100 mg/kg/d | DFO 40 mg/kg/10-12 h/2 d + DFP 75 mg/kg/d | 30-40 mg/kg/d |
| LIC 7- < 15 mg Fe/g dw | 30-40 mg/kg per day, 8-10 h/d, 5 d/wk, SQ | 75-100 mg/kg/d | DFO 40 mg/kg/10-12 h/1-2 d + DFP 75 mg/kg/d | 20-30 mg/kg/d |
| LIC 3- < 7 mg Fe/g dw | 30-40 mg/kg per day, 8-10 h/d, 5 d/wk, SQ | 75 mg/kg/d | Suspend DFO/DFP 75 mg/kg/d | 20-30 mg/kg/d |
| LIC < 3 mg Fe/g dw | Suspend | Suspend | Suspend DFO/Suspend DFP | Suspend |
| Iron intake 0.3-0.5 mg/kg/d | ||||
| LIC ≥ 15 mg Fe/g dw | 40-50 mg/kg per day, 8-10 h/d, 6 or 7 d/wk, SQ | 75-100 mg/kg/d | DFO 40 mg/kg/10-12 h/2 d + DFP 75 mg/kg/d | 30-40 mg/kg/d |
| LIC 7- < 15 mg Fe/g dw | 40-50 mg/kg per day, 8-10 h/d, 6 or 7 d/wk, SQ | 75-100 mg/kg/d | DFO 40 mg/kg/10-12 h/1-2 d + DFP 75 mg/kg/d | 30-40 mg/kg/d |
| LIC 3- < 7 mg Fe/g dw | 30-40 mg/kg per day, 8-10 h/d, 5 d/wk, SQ | 75 mg/kg/d | Suspend DFO/DFP 75 mg/kg/d | 20-30 mg/kg/d |
| LIC < 3 mg Fe/g dw | Suspend | Suspend | Suspend DFO/Suspend DFP | Suspend |
| Iron intake > 0.5 mg/kg/d | ||||
| LIC ≥ 15 mg Fe/g dw | 40-50 mg/kg per day, 8-10 h/d, 6 or 7 d/wk, SQ | 75-100 mg/kg/d | DFO 40 mg/kg/10-12 h/2 d + DFP 75 mg/kg/d | 30-40 mg/kg/d |
| LIC 7- < 15 mg Fe/g dw | 40-50 mg/kg per day, 8-10 h/d, 6 or 7 d/wk, SQ | 75-100 mg/kg/d | DFO 40 mg/kg/10-12 h/2 d + DFP 75 mg/kg/d | 30-40 mg/kg/d |
| LIC 3- < 7 mg Fe/g dw | 30-40 mg/kg per day, 8-10 h/d, 5 d/wk, SQ | 75 mg/kg/d | DFO 40 mg/kg/10-12 h/1 d + DFP 75 mg/kg/d | 20-30 mg/kg/d |
| LIC < 3 mg Fe/g dw | Suspend | Suspend | Suspend DFO/Suspend DFP | Suspend |
| T2* 10- < 20 ms | ||||
| LIC ≥ 15 mg Fe/g dw | 50-60 mg/kg per day, continuous IV | 75-100 mg/kg/d | DFO 40 mg/kg/10-12 h/7 d + DFP 75 mg/kg/d | 40 mg/kg/d |
| LIC 7- < 15 mg Fe/g dw | 40-50 mg/kg per day, 8-10 h/d, 6 or 7 d/wk, SQ | 75-100 mg/kg/d | DFO 40 mg/kg/10-12 h/5 d + DFP 75 mg/kg/d | 30-40 mg/kg/d |
| LIC 3- < 7 mg Fe/g dw | 40-50 mg/kg per day, 8-10 h/d, 6 or 7 d/wk, SQ | 75-100 mg/kg/d | DFO 40 mg/kg/10-12 h/2 d + DFP 75-100 mg/kg/d | 30-40 mg/kg/d |
| LIC < 3 mg Fe/g dw | Adjust to therapeutic index,† monitory safety closely | 75-100 mg/kg/d | DFO 40 mg/kg/10-12 h/1-2 d + DFP 75-100 mg/kg/d/Monitor safety closely | Adjust dose, monitor safety closely |
| T2* < 10 ms | ||||
| LIC ≥ 15 mg Fe/g dw | 50-60 mg/kg per day, continuous IV | Not recommended | DFO 40 mg/kg/10-12 h/7 d + DFP 75-100 mg/kg/d | Not recommended |
| LIC 7- < 15 mg Fe/g dw | 40-50 mg/kg per day, continuous IV | 75-100 mg/kg/d | DFO 40 mg/kg/10-12 h/5-7 d + DFP 75-100 mg/kg/d | 30-40 mg/kg/d |
| LIC 3- < 7 mg Fe/g dw | 40-50 mg/kg per day, continuous IV | 75-100 mg/kg/d | DFO 40 mg/kg/10-12 h/3-5 d + DFP 75-100 mg/kg/d | 30-40 mg/kg/d |
| LIC < 3 mg Fe/g dw | Adjust to therapeutic index,† monitory safety closely | 75-100 mg/kg/d | DFO 40 mg/kg/10-12 h/1-2 d + DFP 75-100 mg/kg/d | Adjust dose, monitor safety closely |
| . | DFO* . | DFP . | DFO + DFP combination . | DFX . |
|---|---|---|---|---|
| T2* ≥ 20 ms | ||||
| Iron intake < 0.3 mg/kg/d | ||||
| LIC ≥ 15 mg Fe/g dw | 40-50 mg/kg per day, 8-10 h/d, 6 or 7 d/wk, SQ | 75-100 mg/kg/d | DFO 40 mg/kg/10-12 h/2 d + DFP 75 mg/kg/d | 30-40 mg/kg/d |
| LIC 7- < 15 mg Fe/g dw | 30-40 mg/kg per day, 8-10 h/d, 5 d/wk, SQ | 75-100 mg/kg/d | DFO 40 mg/kg/10-12 h/1-2 d + DFP 75 mg/kg/d | 20-30 mg/kg/d |
| LIC 3- < 7 mg Fe/g dw | 30-40 mg/kg per day, 8-10 h/d, 5 d/wk, SQ | 75 mg/kg/d | Suspend DFO/DFP 75 mg/kg/d | 20-30 mg/kg/d |
| LIC < 3 mg Fe/g dw | Suspend | Suspend | Suspend DFO/Suspend DFP | Suspend |
| Iron intake 0.3-0.5 mg/kg/d | ||||
| LIC ≥ 15 mg Fe/g dw | 40-50 mg/kg per day, 8-10 h/d, 6 or 7 d/wk, SQ | 75-100 mg/kg/d | DFO 40 mg/kg/10-12 h/2 d + DFP 75 mg/kg/d | 30-40 mg/kg/d |
| LIC 7- < 15 mg Fe/g dw | 40-50 mg/kg per day, 8-10 h/d, 6 or 7 d/wk, SQ | 75-100 mg/kg/d | DFO 40 mg/kg/10-12 h/1-2 d + DFP 75 mg/kg/d | 30-40 mg/kg/d |
| LIC 3- < 7 mg Fe/g dw | 30-40 mg/kg per day, 8-10 h/d, 5 d/wk, SQ | 75 mg/kg/d | Suspend DFO/DFP 75 mg/kg/d | 20-30 mg/kg/d |
| LIC < 3 mg Fe/g dw | Suspend | Suspend | Suspend DFO/Suspend DFP | Suspend |
| Iron intake > 0.5 mg/kg/d | ||||
| LIC ≥ 15 mg Fe/g dw | 40-50 mg/kg per day, 8-10 h/d, 6 or 7 d/wk, SQ | 75-100 mg/kg/d | DFO 40 mg/kg/10-12 h/2 d + DFP 75 mg/kg/d | 30-40 mg/kg/d |
| LIC 7- < 15 mg Fe/g dw | 40-50 mg/kg per day, 8-10 h/d, 6 or 7 d/wk, SQ | 75-100 mg/kg/d | DFO 40 mg/kg/10-12 h/2 d + DFP 75 mg/kg/d | 30-40 mg/kg/d |
| LIC 3- < 7 mg Fe/g dw | 30-40 mg/kg per day, 8-10 h/d, 5 d/wk, SQ | 75 mg/kg/d | DFO 40 mg/kg/10-12 h/1 d + DFP 75 mg/kg/d | 20-30 mg/kg/d |
| LIC < 3 mg Fe/g dw | Suspend | Suspend | Suspend DFO/Suspend DFP | Suspend |
| T2* 10- < 20 ms | ||||
| LIC ≥ 15 mg Fe/g dw | 50-60 mg/kg per day, continuous IV | 75-100 mg/kg/d | DFO 40 mg/kg/10-12 h/7 d + DFP 75 mg/kg/d | 40 mg/kg/d |
| LIC 7- < 15 mg Fe/g dw | 40-50 mg/kg per day, 8-10 h/d, 6 or 7 d/wk, SQ | 75-100 mg/kg/d | DFO 40 mg/kg/10-12 h/5 d + DFP 75 mg/kg/d | 30-40 mg/kg/d |
| LIC 3- < 7 mg Fe/g dw | 40-50 mg/kg per day, 8-10 h/d, 6 or 7 d/wk, SQ | 75-100 mg/kg/d | DFO 40 mg/kg/10-12 h/2 d + DFP 75-100 mg/kg/d | 30-40 mg/kg/d |
| LIC < 3 mg Fe/g dw | Adjust to therapeutic index,† monitory safety closely | 75-100 mg/kg/d | DFO 40 mg/kg/10-12 h/1-2 d + DFP 75-100 mg/kg/d/Monitor safety closely | Adjust dose, monitor safety closely |
| T2* < 10 ms | ||||
| LIC ≥ 15 mg Fe/g dw | 50-60 mg/kg per day, continuous IV | Not recommended | DFO 40 mg/kg/10-12 h/7 d + DFP 75-100 mg/kg/d | Not recommended |
| LIC 7- < 15 mg Fe/g dw | 40-50 mg/kg per day, continuous IV | 75-100 mg/kg/d | DFO 40 mg/kg/10-12 h/5-7 d + DFP 75-100 mg/kg/d | 30-40 mg/kg/d |
| LIC 3- < 7 mg Fe/g dw | 40-50 mg/kg per day, continuous IV | 75-100 mg/kg/d | DFO 40 mg/kg/10-12 h/3-5 d + DFP 75-100 mg/kg/d | 30-40 mg/kg/d |
| LIC < 3 mg Fe/g dw | Adjust to therapeutic index,† monitory safety closely | 75-100 mg/kg/d | DFO 40 mg/kg/10-12 h/1-2 d + DFP 75-100 mg/kg/d | Adjust dose, monitor safety closely |
Modified from Brittenham3 with permission.
dw indicates dry weight; SQ, subcutaneous; and IV, intravenous.
Vitamin C-dose limited to 200 mg/day given orally at the time of infusion.
Therapeutic index = mean daily dose (mg/kg; mean daily dose = actual dose of each infusion × doses/7 days)/ferritin (mg/L). Keep index < 0.025 at all times.