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接受雄激素治疗的范科尼贫血患者出现多发性肝肿瘤和肝紫癜病。

Multiple hepatic tumors and peliosis hepatis in Fanconi's anemia treated with androgens.

作者信息

Shapiro P, Ikeda R M, Ruebner B H, Connors M H, Halsted C C, Abildgaard C F

出版信息

Am J Dis Child. 1977 Oct;131(10):1104-6. doi: 10.1001/archpedi.1977.02120230050009.

Abstract

We report the case of a 13-year-old boy who was known to have Fanconi's anemia for five years. For treatment of this condition he was given androgens and corticosteroids. Two months before his death, severe varicella developed complicated by pneumonia, jaundice, and prolonged fever; all of which resolved during a five-week hospitalization. Three weeks later he died of Clostridium septicum sepsis caused by necrotizing enterocolitis. At autopsy he was found to have multiple hepatocellular neoplasms. A striking feature of the neoplasms was cholestasis. The liver also showed peliosis hepatis. The association of the use of certain androgenic steroids with hepatic neoplasms histologically resembling hepatocarcinomas, but characterized by lack of metastases and apparent reversibility, suggests the desirability of a new nomenclature for these hepatocellular lesions.

摘要

我们报告了一名13岁男孩的病例,他患范科尼贫血已有五年。针对这种疾病,他接受了雄激素和皮质类固醇治疗。在他去世前两个月,出现了严重的水痘,并伴有肺炎、黄疸和持续发热;所有这些症状在为期五周的住院期间都得到了缓解。三周后,他死于坏死性小肠结肠炎引起的败血梭菌败血症。尸检发现他患有多发性肝细胞肿瘤。这些肿瘤的一个显著特征是胆汁淤积。肝脏还显示肝紫癜。使用某些雄激素类固醇与组织学上类似于肝癌但具有无转移和明显可逆性特征的肝脏肿瘤之间的关联,表明需要为这些肝细胞病变采用新的命名法。

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