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中间型地中海贫血患者的脊柱旁髓外造血。

Paraspinal extramedullary hematopoiesis in patients with thalassemia intermedia.

机构信息

Division of Orthopedic Surgery, Department of Surgery, American University of Beirut Medical Center, 11-0236, Riad El Solh, Beirut, 1107 2020, Lebanon.

出版信息

Eur Spine J. 2010 Jun;19(6):871-8. doi: 10.1007/s00586-010-1357-2. Epub 2010 Mar 5.

Abstract

Ineffective erythropoiesis in patients with thalassemia intermedia drives extramedullary hematopoietic tumor formation in several parts of the body. Paraspinal involvement has received increasing attention due to the associated morbidity secondary to spinal cord compression. Although the history and physical examination may help narrow the differential diagnosis, radiographic imaging remains essential to confirm the existence of hematopoietic tissue. Characteristic appearance has been observed mainly on magnetic resonance imaging. Several treatment options have been described, including transfusion therapy, laminectomy, radiotherapy, and the use of fetal hemoglobin inducing agents that decrease the hematopoietic drive. However, the ideal management scheme remains controversial. Until large prospective trials evaluate the efficacy and safety of the available treatment options, both in single and in combination therapy, an individualized approach should be entertained.

摘要

无效的红细胞生成在中间型地中海贫血患者中会导致身体多个部位的髓外造血肿瘤形成。由于脊髓压迫引起的相关发病率增加,脊柱旁受累引起了越来越多的关注。尽管病史和体格检查有助于缩小鉴别诊断范围,但放射影像学检查对于确认造血组织的存在仍然至关重要。主要在磁共振成像上观察到特征性表现。已经描述了几种治疗选择,包括输血治疗、椎板切除术、放疗以及使用降低造血驱动的胎儿血红蛋白诱导剂。然而,理想的治疗方案仍然存在争议。在大型前瞻性试验评估现有治疗选择的疗效和安全性之前,无论是单独使用还是联合使用,都应该采用个体化的方法。

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