Paraspinal Extramedullary Hematopoiesis in a Transfusion-Dependent Beta-Thalassemia Patient: A Case Report

输血依赖型β-地中海贫血患者椎旁髓外造血:病例报告

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Abstract

Extramedullary hematopoiesis (EMH) is a compensatory mechanism in chronic anemias, such as transfusion-dependent beta-thalassemia (TDT), most commonly affecting the liver and spleen. Paraspinal EMH is rare and may lead to spinal cord compression, resulting in neurological deficits. We present a 26-year-old male patient with longstanding TDT who developed progressive bilateral lower limb weakness, pelvic paresthesia, and acute urinary retention. MRI revealed a bilateral paraspinal mass compressing the dural sac and spinal cord, with concurrent severe myocardial and hepatic iron overload. Given the high operative risk and contraindications to surgery and radiotherapy, treatment included hypertransfusion, dual iron chelation therapy, and hydroxyurea. The patient developed hydroxyurea-induced bone marrow aplasia requiring discontinuation of treatment. Over six months, motor and sensory functions improved, bladder function partially recovered, iron markers decreased, and MRI showed regression of the mass. This case highlights the complexities in diagnosing and managing paraspinal EMH in high-risk TDT patients and supports the effectiveness of individualized conservative therapy.

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