A 35-year-old man presented with acute paraplegia. He had a past history of major thalassaemia beta-delta, which was refractory to previous treatment, including splenectomy. Transfusions had failed to adequately increase his haemoglobin concentrations, because of auto-immunisation against Kpb antigen, and he had extramedullary haemopoiesis.
Chest radiography (figure A) showed rib enlargement due to bone marrow hypertrophy and extra-pleural abnormalities. A chest CT scan showed numerous soft-tissue masses (figure B)
with ectopic foci of haemopoiesis and pleural effusions. An MRI of our patient’s spine showed a lobulated mass (figure C) which protruded into the spinal canal and compressed the spinal cord at T7-T10.
Our patient had surgical decompression to remove the lesion; histology (figure D) showed extensive extramedullary haemopoiesis with strong erythroid hyperplasia. A left shift with an increased number of blasts was also seen on histology. After surgery our patient recovered well, and was able to walk.
35岁男性患者，出现急性截瘫。
既往病史：地中海贫血,并行脾切除术。
输血未能有效增加血红蛋白浓度。
髓外造血组织瘤样增生。
（extramedullary hematopoiesis, EMH）
慢性贫血者、CT见双侧脊柱旁多发软组织影。
边缘光整，无囊变、坏死及钙化。
增强轻度强化，冠状位很典型。
髓外造血是一种代偿性增生。
发生在椎管附近或硬膜外者易出现脊髓压迫症状。
部分可有肝脾增大、淋巴结肿大等其他器官代偿性改变。
“肋中肋”为本病特征性征象。
MRI上肿块通常不均质，如果存在脂肪可表现T1高信号。其他表现与慢性贫血有关，包括脾大、骨小梁粗糙和肋骨膨大。
鉴别：
神经源性肿瘤（神经节细胞瘤、交感神经节细胞瘤、神经鞘瘤）：多为单侧、单发，无贫血等继发髓外造血基础疾病，易囊变、坏死、钙化，呈哑铃状
淋巴瘤
胸膜肿瘤（间皮瘤、转移性疾病）