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β地中海贫血/血红蛋白E中髓外造血所致的梗阻性尿路病

Obstructive uropathy due to extramedullary haematopoiesis in beta thalassaemia/haemoglobin E.

作者信息

Intragumtornchai T, Arjhansiri K, Posayachinda M, Kasantikul V

机构信息

Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.

出版信息

Postgrad Med J. 1993 Jan;69(807):75-7. doi: 10.1136/pgmj.69.807.75.

Abstract

An 18 year old woman with beta thalassaemia/haemoglobin E developed a large pelvic tumour resulting in bilateral obstructive uropathy. Technetium-99m sulphur colloid marrow image, computed tomographic scan of the abdomen and needle biopsy of the mass confirmed the diagnosis of extramedullary haematopoiesis. Although radiation is the treatment of choice for decompression, the mass in this patient did not respond satisfactorily due to its multiple area of tumour autoinfarction. Obstructive uropathy due to extramedullary erythropoiesis has not to our knowledge been previously described.

摘要

一名患有β地中海贫血/血红蛋白E的18岁女性出现了一个巨大的盆腔肿瘤,导致双侧梗阻性尿路病。锝-99m硫胶体骨髓显像、腹部计算机断层扫描及肿块穿刺活检确诊为髓外造血。尽管放射治疗是减压的首选治疗方法,但由于该患者肿瘤存在多个自动梗死区域,肿块对此治疗反应不令人满意。据我们所知,此前尚未有关于髓外红细胞生成导致梗阻性尿路病的描述。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c79c/2399576/147cd9a70aed/postmedj00049-0079-a.jpg

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