V. De Sanctis et al., Microstructural analysis of severe bone lesions in seven thalassemic patients treated with deferoxamine, CALCIF TIS, 67(2), 2000, pp. 128-133
Osteochondrodystrophic lesions, mainly affecting long bane metaphyses, can
be radiologically evident in homozygotic thalassemic patients treated with
deferoxamine, and their incidence rate varies among authors. The clinical a
nd radiological appearance of these lesions is described in the literature,
but microstructural data are still lacking. The aim of our research was to
evaluate the microstructure of five tibial biopsy specimens from thalassem
ic patients with bone lesions (5 cases out of 180 patients followed for the
last 10 years, i.e., 2.8%) and two bone biopsy specimens from thalassemic
patients with no radiological alteration of the long bones. As control, bon
e tissue taken from autoptic tibiae of two subjects with no skeletal pathol
ogy was used.
Using microradiography and X-ray diffraction (XRD), we found a reduced and
irregular mineralization of the bone (compared with controls) in thalassemi
c subjects. Bone tissue microhardness was also significantly reduced. Never
theless, bone apatite lattice was unaltered and no 'foreign' crystallograph
ic phase was recorded by XRD. In conclusion, all the patients shared a simi
lar picture of abnormal bone, even with no radiological evidence of lesion.