In 32 patients with thalassemia the spectrum of rib changes, including widening, osteoporosis, localized lucencies, cortical erosions, "rib-within-a-rib" appearance, subcortical lucency, and extramedullary hematopoiesis, is reviewed. This material is supplemented by the radiographic and computed tomographic findings in a specimen of spine, posterior ribs, and extramedullary hematopoietic masses. The relevant literature on thalassemia, including two cases of "costal osteoma," is reviewed. A mechanism is offered to explain the various rib changes based on (a) the relationship of the proliferating marrow to the medulla, cortex, and periosteum, (b) the patient's age at the onset of a transfusion regimen, and (c) the type of transfusion regimen used.
A spectrum of rib changes was seen in 32 patients with thalassemia. Eleven patients had normal ribs, and 21 patients demonstrated at least one abnormality. The rib changes in patients who had been treated with a low transfusion regimen (designed to maintain the hemoglobin level at 5-6 g/100 ml) were compared with the changes in patients who had been treated with a hypertransfusion regimen (designed to maintain the hemoglobin level above 9.4-19 g/100 ml). Ten of the 11 patients (91%) with normal ribs were younger than five years when first hypertransfused, while only three of the 21 patients (14%) with abnormal ribs were younger than five. The type of rib abnormality was related to the age of onset of the low transfusion regimen. Those patients with abnormal but nonwidened ribs had been started on the low transfusion regimen earlier than those patients with the same abnormality and widened ribs. Fifteen of the 21 patients showed regression of the abnormal findings; approximately two thirds of the rib abnormalities did not regress until after the onset of a hypertransfusion regimen.
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