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因地中海贫血导致的髓外造血所致的复发性脊髓压迫症:病例报告。

Recurrent spinal cord compression due to extramedullary hematopoiesis in thalassemia patient: Case report.

机构信息

Internal Medicine Department, Hamad Medical Corporation, Doha, Qatar.

Radiology Department, Hamad Medical Corporation, Doha, Qatar.

出版信息

Medicine (Baltimore). 2022 Jun 24;101(25):e29334. doi: 10.1097/MD.0000000000029334.

Abstract

INTRODUCTION

Spinal cord compression secondary to extramedullary hematopoiesis (EMH) is a rare condition. Variable treatment options have been reported with different efficacy and recurrence rate. Due to its rarity, no clear optimal management guidelines have been established yet.

PATIENT CONCERNS AND DIAGNOSIS

We report a recurrence of spinal cord compression secondary to EMH in a 19-year-old male, with a background of transfusion-dependent beta-thalassemia on luspatercept, who presented with weakness in both lower limbs.

INTERVENTIONS AND OUTCOMES

He was treated successfully both times with radiotherapy.

CONCLUSION

Early diagnosis and management of EMH compressing the spinal cord are essential to prevent permanent neurological damage. Diagnosis should be suspected based on the clinical presentation and magnetic resonance imaging findings in a patient with a history of ineffective hemopoiesis. Treatment option remains controversial. Radiotherapy option seems effective, even in recurrent cases, and valid, particularly for those at high risk of surgery or who do not prefer it.

摘要

简介

继发于髓外造血(EMH)的脊髓压迫是一种罕见的情况。已有报道称,不同的治疗方法具有不同的疗效和复发率。由于其罕见性,尚未建立明确的最佳治疗指南。

病例介绍

我们报告了一例 19 岁男性患者,因输血依赖型β地中海贫血,使用 luspatercept 治疗后,继发 EMH 导致脊髓压迫复发,表现为双下肢无力。

干预措施和结果

他两次均成功接受了放射治疗。

结论

早期诊断和治疗压迫脊髓的 EMH 对于预防永久性神经损伤至关重要。对于有无效造血病史的患者,基于临床表现和磁共振成像结果,应怀疑诊断。治疗方案仍存在争议。放射治疗方案似乎有效,即使在复发病例中也是如此,而且对于那些手术风险高或不喜欢手术的患者来说是合理的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a235/9276436/9deb1d2168ad/medi-101-e29334-g001.jpg

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