Premature epiphyseal fusions in beta thalassaemia

Thalassemia patients are now living longer due to better transfusion methods and diagnostic awareness. To see whether this longevity is associated with orthopaedic disability, especially physeal growth defects, we examined 105 patients aged between 5-25 years for evidence of clinically detectable premature epiphyseal fusions (PEF). Ours is a center focussed on transfusion dependent beta thalassemia (TDBT) patient management, and so detailed transfusion records related to age at first transfusion, regularity of transfusions and pre-transfusional haemoglobin (Hb) levels were available. Five (4.7%) patients had deformities or limb length discrepancies, which lead to the detection of PEF. All patients with PEF had pre transfusion haemoglobin levels of less than 8 gm/dL. On comparing with the literature, we found that the prevalence of clinically detectable PEF in TDBT patients has decreased with better blood transfusion regimes. Though the pathogenesis of PEF is yet to be conclusively established, it is apparent that better control of the disease to maintain pre-transfusional haemoglobin levels consistently above 8 gm/dL in the first decade, can decrease the occurrence of PEF.