Skeletal changes in thalassemia major

The authors clinically and radiographically examined 72 patients with homozygous beta thalassemia. The clinical data were compared to the degree of osteoporosis calculated by Singh's method. The results indicate a high incidence of skeletal changes in patients with thalassemia, including lower limb-length discrepancy (16.6%), upper limb-length discrepancy (5.5%), axial deviation of the limbs (8.3%), osteochondrosis (2.7%), and osteopenia (25%). Based on their observations, the authors identify skeletal changes of adulthood (osteopenia) and childhood (limb-length discrepancy, axial deviation, osteochondrosis). The authors hypothesize that osteoporotic changes are caused principally by hyperplasia of the bone marrow, which widens the medullary space and increases intramedullary pressure, leading eventually to osteoporosis.