SPINAL-CORD COMPRESSION DUE TO EXTRAMEDULLARY HEMATOPOIESIS IN BETA-THALASSEMIA-INTERMEDIA

Background: Extramedullary hematopoiesis (EMH) occurs in many disorder s, including thalassemias and other hemoglobinopathies, and commonly p resents in the spleen and liver. We present a case of spinal cord comp ression in a patient with beta-thalassemia intermedia, and review the literature and available treatment options. Patient and Methods: A 35- year-old black female with beta-thalassemia intermedia presented with a 3-week history of back pain and lower extremity weakness. Neurologic examination was consistent with spinal cord compression, and gadolini um enhanced magnetic resonance imaging (MRI) confirmed this diagnosis. She was given intravenous steroids and radiotherapy was begun in 200 cGy fractions to a total dose of 2000 cGy, Results: At the completion of radiotherapy the patient was ambulatory with mild residual weakness . MRI scans 16 months later showed smaller, but persistent masses, and she remains asymptomatic 5 years from her diagnosis. Conclusion: Reco gnition of spinal cord EMH requires prompt physical examination and MR I for accurate diagnosis. EMH can be managed with radiation, surgery, transfusions, or a combination of these therapies. Radiation in conser vative doses of (750-3500 cGy) is non-invasive, avoids the surgical ri sks of potentially severe hemorrhage and incomplete resection, and has a high complete remission rate in the majority of patients. Relapse r ates are moderate (37.5%), but retreatment provides excellent chance f or second remission. (C) 1998 Elsevier Science Inc.