Spinal cord compression in homozygous beta-thalassemia intermedia.

Symptomatic spinal cord compression caused by an epidural mass of extramedullary hematopoietic tissue in patients with beta-thalassemia is a rare occurrence, that becomes exceptional in childhood and adolescence. The literature is not uniform about the optimal treatment of these patients and different modes of therapy have been proposed so far, including surgical excision or decompressing laminectomy followed by local irradiation and/or hypertransfusional regimens. We report the successful treatment of such a complication in an adolescent with homozygous beta-thalassemia by surgery followed by repeated blood transfusions aimed at maintaining hematocrit at normal levels.