Effective non-surgical management of spinal cord compression from extra-medullary hematopoiesis with radiotherapy in thalassemia intermedia: a case report

放射治疗有效治疗中间型地中海贫血髓外造血引起的脊髓压迫:一例病例报告

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Abstract

Spinal cord compression (SCC) secondary to extramedullary hematopoiesis (EMH) represents a rare but critical manifestation in patients with hematologic conditions, including β-thalassemia intermedia (β-TI). While there is no standard guideline due to the rarity of EMH-induced SCC, this case report documents successful management with radiotherapy while avoiding high-risk surgical intervention. The patient was a 17-year-old male with β-TI who developed debilitating SCC attributable to a paraspinal EMH mass. Due to concerns regarding surgical morbidity, a 26Gy 13-session course of radiation was administered alongside adjunctive blood transfusions, Hydroxyurea, and Glucocorticoids. Marked improvement and subsequent resolution of neurological deficits occurred rapidly following treatment, with sustained regression of the EMH mass over 18 months of follow-up. The case demonstrates that radiotherapy can be an effective primary treatment for EMH-induced SCC in β-TI, emphasizing its safety and efficacy when immediate and significant clinical response is required.

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