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巨大的髓外造血是先天性红细胞生成异常性贫血的一种并不罕见的并发症。

Bulky extramedullary hematopoiesis is not a rare complication of congenital dyserythropoietic anemia.

作者信息

Heimpel Hermann, Dührsen Ulrich, Hofbauer P, Rigamonti-Wermlinger V, Kreuser Ernst-Dietrich, Schwarz Klaus, Solenthaler Max, Pauls S

机构信息

Medizinische Klinik III, Universitätsklinikum Ulm, Ulm, Germany.

出版信息

Ann Hematol. 2009 Oct;88(10):937-41. doi: 10.1007/s00277-009-0735-5. Epub 2009 Mar 31.

Abstract

Bulky extramedullary hematopoiesis, usually detected in the thorax by imaging techniques, is a well-known complication in many types of congenital anemias. Here, we describe 12 cases of congenital dyserythropoietic anemia with extramedullary hematopoiesis which was always located in the paravertebral space of the thoracic spine and in other paraspinal regions in a few cases. All bulks were originally detected in chest radiographs and confirmed by imaging techniques such as computed tomography and/or magnetic resonance imaging. In some cases, thoracotomy was performed for suspected malignancy. Although the true prevalence is not known, paravertebral masses in patients with CDA of any type are not uncommon and should be the first differential diagnosis considered when masses adjacent to the spine are detected in this disorder.

摘要

大量髓外造血通常通过影像学技术在胸部被检测到,是多种先天性贫血中一种众所周知的并发症。在此,我们描述了12例先天性红细胞生成异常性贫血合并髓外造血的病例,其髓外造血总是位于胸椎的椎旁间隙,少数病例位于其他脊柱旁区域。所有肿块最初均在胸部X线片上被检测到,并通过计算机断层扫描和/或磁共振成像等影像学技术得以证实。在某些病例中,因怀疑为恶性肿瘤而进行了开胸手术。尽管真实患病率尚不清楚,但任何类型的先天性红细胞生成异常性贫血患者出现椎旁肿块并不罕见,当在该疾病中检测到脊柱旁肿块时,应首先考虑进行鉴别诊断。

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