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Focal hepatic lesion in Gaucher disease mimicking hepatocellular carcinoma: A case report
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摘要: <正>戈谢病（Gaucher disease,GD）是一种罕见的常染色体隐性遗传病,该病由于葡糖脑苷脂酶（glucerebrosidase,GBA）缺乏引起底物葡糖脑苷脂聚集于巨噬细胞溶酶体中而发病。脂质过载的巨噬细胞,即戈谢细胞主要浸润脾、肝脏和骨髓。临床表现为贫血、血小板少、脾大、肝大以及骨骼受累,然而不同患者其临床表现差异显著[1-3]。近年来,GD患者局灶性肝损伤影像学多样性,引起人们关注[4-6]。
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Key words:
- Gaucher disease /
- focal hepatic lesion /
- carcinoma,hepatocellular
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[1] ESSABAR L,MESKINI T,LAMALMI N,et al. Gaucher’s disease:Report of 11 cases with review of literature[J]. Pan Afr Med J,2015,20:18. [2] MISTRY PK,CAPPELLINI MD,LUKINA E,et al. A reappraisal of Gaucher disease-diagnosis and disease management algorithms[J]. Am J Hematol,2011,86(1):110-115. [3] STIRNEMANN J,BELMATOUG N,CAMOU F,et al. A review of Gaucher disease pathophysiology,clinical presentation and treatments[J]. Int J Mol Sci,2017,18(2):e441. [4] REGENBOOG M,BOHTE AE,SOMERS I,et al. Image characteristics of focal spleen and hepatic lesions in type 1 Gaucher disease[J]. Blood Cells Mol Dis,2016,60:49-57. [5] POLL LW,VOM DAHL S. Image of the month. Hepatic Gaucheroma mimicking focal nodular hyperplasia[J]. Hepatology,2009,50(3):985-986. [6] REGENBOOG M,van DUSSEN L,VERHEIJ J,et al. Hepatocellular carcinoma in Gaucher disease:An international case series[J]. J Inherit Metab Dis,2018,41(5):819-827. -
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