Fareed Shehab, Soliman Ashraf T, De Sanctis Vincenzo, Kohla Samah, Soliman Dina, Khirfan Diala, Tambuerello Adriana, Talaat Mohamed, Nashwan Abdulqadir, Caparrotti Palmira, Yassin Mohamed A
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Acta Biomed. 2017 Aug 23;88(2):237-242. doi: 10.23750/abm.v88i2.6221.
We report a case of a thalassemia major male patient with back pain associated to severe weakness in lower extremities resulting in the ability to ambulate only with assistance. An urgent magnetic resonance imaging (MRI) of thoracic and lumbosacral spine was requested. A posterior intraspinal extradural mass lesion compressing the spinal cord at the level of thoracic T5-8 was present, suggesting an extramedullary hematopoietic centre, compressing the spinal cord. He was treated successfully with thalassemia major alone. The patient was treated with blood transfusion, dexamethasone, morphine and paracetamol, followed by radiotherapy in 10 fractions to the spine (daily fraction of 2Gy from T3 to T9, total dose 20 Gy). His pain and neurologic examination quickly improved. A new MRI of the spine, one week after radiotherapy, showed an improvement of the extramedullary hematopoietic mass compression. In conclusion, EMH should be considered in every patient with ineffective erythropoiesis and spinal cord symptoms. MRI is the most effective method of demonstrating EMH. The rapid recognition and treatment can dramatically alleviate symptoms. There is still considerable controversy regarding indications, benefits, and risks of each of modality of treatment due to the infrequency of this disorder.
我们报告一例重型地中海贫血男性患者,其背痛伴双下肢严重无力,导致只能在他人协助下行走。遂要求对胸腰骶椎进行紧急磁共振成像(MRI)检查。结果显示在胸段T5 - 8水平存在一个压迫脊髓的椎管内硬膜外肿块病变,提示为一个压迫脊髓的髓外造血中心。仅采用重型地中海贫血的治疗方法,他就获得了成功治疗。该患者接受了输血、地塞米松、吗啡和对乙酰氨基酚治疗,随后对脊柱进行了10次分割放疗(从T3至T9每日分割剂量为2Gy,总剂量20Gy)。他的疼痛和神经学检查迅速改善。放疗一周后脊柱的新MRI显示髓外造血肿块压迫情况有所改善。总之,对于每一位红细胞生成无效且有脊髓症状的患者都应考虑髓外造血(EMH)。MRI是显示EMH最有效的方法。快速识别和治疗可显著缓解症状。由于这种疾病罕见,关于每种治疗方式的适应证、益处和风险仍存在相当大的争议。