Sohawon Dharamraj, Lau Kenneth K, Lau Theodore, Bowden Donald K
Department of Surgery, Austin Health, Nursing and Health Sciences Centre for Green Chemistry, Monash University, Queensland, Australia.
J Med Imaging Radiat Oncol. 2012 Oct;56(5):538-44. doi: 10.1111/j.1754-9485.2012.02397.x. Epub 2012 Jun 25.
Extra-medullary haematopoiesis (EMH) occurs in many haematological disorders and is secondary to insufficient bone marrow function or ineffective erythropoiesis. It usually presents as haematopoietic masses in several typical and atypical body locations. This pictorial review briefly discusses the common EMH locations encountered in clinical practice, including the liver, spleen, lymph nodes and paravertebral regions. Unusual presentation as focal hepatic and splenic masses is also discussed. Some atypical EMH locations are then presented together with their pathophysiology and relevant illustrations. These include EMH in the intra-spinal canal, pre-sacral region, nasopharynx and paranasal sinuses. Intra-spinal EMH can cause cord compression and present with neurological symptoms. In these cases, urgent treatment may be required. Haematopoietic masses in the other atypical locations can present with obstructive symptoms or may be diagnosed incidentally on imaging. EMH in unusual locations need to be monitored with follow-up imaging to ensure their stability. In some circumstances, tissue biopsy is appropriate to confirm the diagnosis and exclude other sinister pathology (e.g. malignancy). Treatment is only required where symptoms are present. Management options include blood transfusion, radiotherapy, hydroxyurea or surgical resection in selected cases.
髓外造血(EMH)发生于多种血液系统疾病,继发于骨髓功能不足或无效红细胞生成。它通常表现为在几个典型和非典型身体部位出现造血性肿块。本图像综述简要讨论了临床实践中常见的EMH部位,包括肝脏、脾脏、淋巴结和椎旁区域。还讨论了表现为肝脏和脾脏局灶性肿块的不寻常情况。然后介绍了一些非典型EMH部位及其病理生理学和相关图示。这些部位包括椎管内、骶前区域、鼻咽部和鼻窦的EMH。椎管内EMH可导致脊髓压迫并出现神经症状。在这些情况下,可能需要紧急治疗。其他非典型部位的造血性肿块可出现梗阻症状,或在影像学检查时偶然被诊断。非典型部位的EMH需要通过后续影像学检查进行监测,以确保其稳定性。在某些情况下,组织活检有助于确诊并排除其他严重病变(如恶性肿瘤)。仅在出现症状时才需要治疗。治疗选择包括输血、放疗、羟基脲或在特定病例中进行手术切除。