A. Mangiagli et al., Treatment with deferiprone (L1) in a thalassemic patient with bone lesionsdue to desferrioxamine, J PED END M, 13(6), 2000, pp. 677-680
A male thalassemia major patient who developed bone and cartilage abnormali
ties with a standard dose of desferrioxamine (DFX) given subcutaneously fro
m the age of 4 years was treated with the oral iron chelator deferiprone (L
1). During L1 treatment an improvement of genu valgum, evidence of healing
and filling in of bone at the periphery of knee metaphysis and improvement
in growth velocity were observed. However, the sitting height had decreased
further, confirming the irreversibility of platyspondylosis, so affecting
the near final standing height (156.8 cm) which was below the mid-parental
height (168 cm). Prospective studies are warranted to determine the effect
of different iron chelators on the bone metabolism of patients with thalass
emia.