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异基因骨髓移植后再生障碍性贫血合并继发性血色素沉着症

[Aplastic anemia complicated with secondary hemochromatosis after allogenic bone marrow transplantation].

作者信息

Oguchi T, Kitano K, Kobayashi H, Saito H, Okamoto S, Kiyosawa K

机构信息

Second Department of Internal Medicine, Shinshu University School of Medicine.

出版信息

Rinsho Ketsueki. 1995 Oct;36(10):1188-92.

PMID:8531329
Abstract

We report a case of aplastic anemia complicated with secondary hemochromatosis after allogenic bone marrow transplantation (BMT). A 29-year-old man was diagnosed as having aplastic anemia at the age of 8. At the age of 28, BMT was performed from his HLA-identical sister. Total volume of blood transfusion before BMT was about 28,000 ml, and in three months after BMT was 8,000 ml. The transplantation was successful, but one month after BMT, dry eyes, skin pigmentation and hepatomegaly appeared. Serum bile duct enzymes and ferritin also increased remarkably. Moreover after thirteen months, glucose tolerance impaired seriously. Abdominal computed tomography (CT) revealed atrophic pancreas and an increased CT density in the liver and the tail of the pancreas. A large amount of iron deposition were also found in liver and stomach biopsy specimens. We concluded that diabetes mellitus was due to secondary hemochtomatosis in the present case. There is a possibility that tissue damage due to iron deposits may have been accelerated through BMT in this patient with a history of many blood transfusions.

摘要

我们报告一例异基因骨髓移植(BMT)后发生再生障碍性贫血并继发血色素沉着症的病例。一名29岁男性在8岁时被诊断为再生障碍性贫血。28岁时,接受了来自其 HLA 相同的姐姐的骨髓移植。骨髓移植前的输血总量约为28,000毫升,骨髓移植后三个月为8,000毫升。移植成功,但骨髓移植后一个月,出现了干眼症、皮肤色素沉着和肝肿大。血清胆管酶和铁蛋白也显著升高。此外,13个月后,葡萄糖耐量严重受损。腹部计算机断层扫描(CT)显示胰腺萎缩,肝脏和胰腺尾部的CT密度增加。在肝脏和胃活检标本中也发现大量铁沉积。我们得出结论,在本病例中糖尿病是由继发性血色素沉着症引起的。对于这名有多次输血史的患者,有可能骨髓移植加速了铁沉积导致的组织损伤。

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引用本文的文献

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Iron chelation therapy in the myelodysplastic syndromes and aplastic anemia: a review of experience in South Korea.骨髓增生异常综合征和再生障碍性贫血中的铁螯合疗法:韩国经验综述
Int J Hematol. 2008 Jul;88(1):16-23. doi: 10.1007/s12185-008-0117-0. Epub 2008 Jul 5.