Research Article

The Management of Iron Chelation Therapy: Preliminary Data from a National Registry of Thalassaemic Patients

Table 4

Chelators use recommendations (SmPCs and Guidelines).

Age: >2 and <6 ySmPCs information: the only approved drug in this group of age is DFO. Oral chelators can be used if DFO is refused, inadequate, or contraindicated. No data or few data are available in children < 6 years for oral chelators (DFP and DFX). Few data between 6 and 10 years for DFP.
UK guideline: DFO is recommended as first-line treatment, DFX as second line.
TIF guideline: DFO is recommended as first-line treatment, oral chelators as second line. DFP above 10 years.
Italian guideline: DFO is recommended as first-line treatment. The use of oral chelators as first-line therapy should be considered investigational and should only be performed within clinical trials or registries.

Age: children >6 y and adultsSmPCs information: DFO and DFX approved. DFP approved if DFO is refused, inadequate, or contraindicated. DFP demonstrated to be superior to deferoxamine in decreasing cardiac iron load. (Ferriprox updated SmPC, 2010).
UK guideline: DFO as first-line treatment, DFX as second line, DFP (in combination with DFO) if abnormal cardiac function (T2* or ECHO or clinical symptoms).
TIF guideline: Oral chelators can be used if DFO is refused, inadequate, or contraindicated. Off label use of DFP should be avoided. For patients with very high levels of heart iron or cardiac dysfunction, 24-hour treatment with deferoxamine and daily therapy with deferiprone should be considered.
Italian guideline: DFO is recommended as first-line treatment where oral chelators therapy should be considered investigational and should only be performed within clinical trials or registries.