Moldvay J, Schaff Z, Lapis K
1st Institute of Pathology and Experimental Cancer Research, Semmelweis Medical University, Budapest, Hungary.
Zentralbl Pathol. 1991;137(2):167-70.
The case of an 11-year-old boy is reported who was known to have Fanconi's anemia for 3 years and was treated with androgens, corticosteroids and transfusions. Two weeks before his death he was readmitted because of aplastic crisis with septicemia and marked abnormalities in liver function and died of hemorrhagic bronchopneumonia. At autopsy peliosis and multiple hepatic tumors were found which histologically proved to be well-differentiated hepatocellular carcinoma. This case contributes to the previous observations that non-metastasizing hepatic neoplasms and peliosis can develop in patients with androgen- and corticosteroid-treated Fanconi's anemia.
报告了一名11岁男孩的病例,该男孩患范科尼贫血3年,接受雄激素、皮质类固醇和输血治疗。在他去世前两周,因再生障碍危象合并败血症及肝功能明显异常而再次入院,最终死于出血性支气管肺炎。尸检发现肝紫癜和多个肝肿瘤,组织学检查证实为高分化肝细胞癌。该病例支持了之前的观察结果,即接受雄激素和皮质类固醇治疗的范科尼贫血患者可能会发生非转移性肝肿瘤和肝紫癜。