Bukhari Syed Sarmad, Junaid Muhammad, Rashid Mamoon Ur
Department of General Surgery, Khyber Teaching Hospital, Peshawar, Pakistan.
Department of Neurosurgery, Pakistan Navy Ship Shifa Hospital, Karachi, Pakistan.
Surg Neurol Int. 2016 Mar 2;7(Suppl 5):S148-52. doi: 10.4103/2152-7806.177891. eCollection 2016.
Extramedullary hematopoiesis (EMH) refers to hematopoiesis outside of the medulla of the bone. Chronic anemia states such as thalassemia can cause hematopoietic tissue to expand in certain locations. We report a case of spinal cord compression due to recurrent spinal epidural EMH, which was treated with a combination of surgery and radiotherapy. Pakistan has one of the highest incidence and prevalence of thalassemia in the world. We describe published literature on diagnosis and management of such cases.
An 18-year-old male presented with bilateral lower limb paresis. He was a known case of homozygous beta thalassemia major. He had undergone surgery for spinal cord compression due to EMH 4 months prior to presentation. Symptom resolution was followed by deterioration 5 days later. He was operated again at our hospital with complete resection of the mass. He underwent local radiotherapy to prevent recurrence. At 2 years follow-up, he showed complete resolution of symptoms. Follow-up imaging demonstrated no residual mass.
The possibility of EMH should be considered in every patient with ineffective erythropoiesis as a cause of spinal cord compression. Treatment of such cases is usually done with blood transfusions, which can reduce the hematopoietic drive for EMH. Other options include surgery, hydroxyurea, radiotherapy, or a combination of these on a case to case basis.
髓外造血（EMH）是指在骨髓以外的部位进行造血。诸如地中海贫血等慢性贫血状态可导致造血组织在某些部位扩张。我们报告一例因复发性脊髓硬膜外髓外造血导致脊髓压迫的病例，该病例采用手术和放疗联合治疗。巴基斯坦是世界上地中海贫血发病率和患病率最高的国家之一。我们描述了关于此类病例诊断和管理的已发表文献。
一名18岁男性出现双侧下肢麻痹。他是已知的重型纯合子β地中海贫血患者。在就诊前4个月，他因髓外造血导致脊髓压迫接受了手术。症状缓解5天后病情恶化。他在我院再次接受手术，肿瘤被完全切除。他接受了局部放疗以预防复发。在2年的随访中，他的症状完全缓解。随访影像学检查未显示残留肿块。
对于每一位因无效红细胞生成导致脊髓压迫的患者，都应考虑髓外造血的可能性。此类病例的治疗通常采用输血，这可以减少髓外造血的造血驱动力。其他选择包括手术、羟基脲、放疗或根据具体情况将这些方法联合使用。
