Evrard C, Pichereau D, Alsweis S, Martel B, Galateau F, Houtteville J P, Benazza A, Leporrier M
Service de Chirurgie Thoracique et Cardio-vasculaire, CHU de Caen.
Ann Chir. 1994;48(3):284-93.
The authors report a case of extramedullary haematopoiesis, presenting in the form of several tumor masses in the posterior mediastinum and in the vertebral canal. The patient presented with oedema in his lower limbs due to compression of the inferior vena cava by the mediastinal masses. The asymptomatic mass in the vertebral canal was detected by magnetic resonance imaging. The patient was first operated on to avoid spinal cord compression by the tumor and subsequently to relieve compression of the inferior vena cava. The masses removed from the vertebral canal and the mediastinum were found on histologic examination to be extramedullary haematopoiesis. The cause of the disease was unknown in this case. Such disease is often disclosed in the course of hemolytic anaemias or other medullar deficiency diseases as a compensatory phenomenon when the bone marrow fails. An other explanation is that it is a manifestation of a myeloproliferative disorder in which heterotopic pluripotential cells are transformed into haematopoietic cells. The authors review 88 cases reported in the world literature.
作者报告了一例髓外造血病例,表现为后纵隔和椎管内的多个肿瘤样肿块。患者因纵隔肿块压迫下腔静脉而出现下肢水肿。椎管内无症状肿块通过磁共振成像检测到。患者首先接受手术以避免肿瘤压迫脊髓,随后缓解下腔静脉受压。经组织学检查发现,从椎管和纵隔切除的肿块为髓外造血。该病例病因不明。这种疾病常在溶血性贫血或其他骨髓缺乏性疾病过程中作为骨髓功能衰竭时的一种代偿现象而被发现。另一种解释是,它是骨髓增殖性疾病的一种表现,其中异位多能细胞转化为造血细胞。作者回顾了世界文献中报道的88例病例。