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一名β地中海贫血患者的肾上腺髓外造血肿瘤。

Adrenal extramedullary hematopoietic tumor in a patient with beta-thalassemia.

作者信息

Chuang C K, Chu S H, Fang J T, Wu J H

机构信息

Department of Surgery, Chang Gung Memorial Hospital, Chang Gung College of Medicine & Technology, Taipei, Taiwan.

出版信息

J Formos Med Assoc. 1998 Jun;97(6):431-3.

PMID:9650475
Abstract

We report a case of adrenal extramedullary hematopoiesis in a 27-year-old woman with beta-thalassemia. She underwent cholecystectomy for gallstones and splenectomy for splenomegaly at the age of 20 years. In January 1996, she was admitted to our hospital because of a palpable nontender mass in the right subcostal area. Ultrasound, computed tomography, and magnetic resonance imaging disclosed a right suprarenal mass 7.5 x 5.8 cm in size. Surgical exploration and biopsy confirmed the diagnosis of extramedullary hematopoietic (EMH) tumor. The tumor was left in situ. Subsequent follow-up showed no increase in the size of the tumor and no progression of anemia. The patient remained asymptomatic, and no additional EMH tumor developed. Surgery or radiotherapy should be performed only when EMH tumors cause symptoms, such as during spinal cord compression. Adrenal EMH tumors should be considered in thalassemia patients with an adrenal mass, to avoid unnecessary surgical procedures.

摘要

我们报告一例27岁患有β地中海贫血的女性肾上腺髓外造血病例。她20岁时因胆结石接受了胆囊切除术,因脾肿大接受了脾切除术。1996年1月,她因右肋下区可触及无痛性肿块入住我院。超声、计算机断层扫描和磁共振成像显示右肾上腺肿块,大小为7.5×5.8厘米。手术探查和活检确诊为髓外造血(EMH)肿瘤。肿瘤未做处理。随后的随访显示肿瘤大小未增加,贫血也未进展。患者一直无症状,也未出现其他EMH肿瘤。仅当EMH肿瘤引起症状时,如脊髓受压时,才应进行手术或放疗。对于有肾上腺肿块的地中海贫血患者,应考虑肾上腺EMH肿瘤,以避免不必要的手术。

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