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一名依赖输血的β地中海贫血患者的椎旁髓外造血:病例报告

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

作者信息

Rawashdeh Zaina, Rawashdeh Ahmad, Al-Rahamnah Rama A, Elghoul Yara I

机构信息

Faculty of Medicine, University of Jordan, Amman, JOR.

General Practice, Jordan University Hospital, Amman, JOR.

出版信息

Cureus. 2025 Jul 4;17(7):e87271. doi: 10.7759/cureus.87271. eCollection 2025 Jul.

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.

摘要

髓外造血(EMH)是慢性贫血中的一种代偿机制,如依赖输血的β地中海贫血(TDT),最常累及肝脏和脾脏。椎旁EMH罕见,可能导致脊髓受压,进而引起神经功能缺损。我们报告一名26岁的长期患有TDT的男性患者,该患者出现进行性双侧下肢无力、盆腔感觉异常和急性尿潴留。MRI显示双侧椎旁肿块压迫硬脊膜囊和脊髓,同时伴有严重的心肌和肝脏铁过载。鉴于手术风险高以及手术和放疗的禁忌证,治疗包括强化输血、双重铁螯合疗法和羟基脲。患者出现羟基脲诱导的骨髓再生障碍,需要停止治疗。在六个月的时间里,运动和感觉功能改善,膀胱功能部分恢复,铁指标下降,MRI显示肿块消退。该病例突出了高危TDT患者椎旁EMH诊断和管理的复杂性,并支持个体化保守治疗的有效性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/70a3/12318370/9d19e43219d8/cureus-0017-00000087271-i01.jpg

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