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Extramedullary haemopoiesis and spinal cord compression.

机译:髓外造血和脊髓压迫。

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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 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.
机译:一名35岁的男子患有急性截瘫。他有严重地中海贫血β-δ的病史,对以前的治疗(包括脾切除术)无效。由于针对Kpb抗原的自身免疫,输血未能充分增加他的血红蛋白浓度,而且他患有髓外造血。胸部X线照片(图A)显示,由于骨髓肥大和胸膜外异常,肋骨增大。胸部CT显示大量软组织肿块(图B),并伴有异位性造血和胸腔积液。我们患者脊柱的MRI显示有一个小叶状肿块(图C),该肿块突出到椎管内并在T7-T10处压缩了脊髓。我们的患者进行了手术减压以去除病变;组织学检查(图D)显示广泛的髓外造血,伴有强烈的红系增生。在组织学上也观察到左移,胚泡数目增加。手术后,我们的患者康复良好,能够行走。

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