Ahmed Moiz, Kesavan Mayurathan, Jilani Basmah N, Ahmed Saba, Deeb Liliane
Department of Internal Medicine, Staten Island University Hospital, Northwell Health, Staten Island, NY, USA.
Department of Gastroenterology, Staten Island University Hospital, Northwell Health, Staten Island, NY, USA.
Cureus. 2016 Jun 3;8(6):e629. doi: 10.7759/cureus.629.
Systemic mastocytosis is a rare infiltrative disease involving the skin, bone marrow, digestive system, and liver. We report a case of a 59-year-old male who presented with a massive variceal bleed without any evidence of cirrhosis; however was later found to have severe perisinusoidal fibrosis with mast cells in portal tracts on liver biopsy and hypercellular mast cell infiltrated bone marrow. This rare case describes an out-of-the-ordinary reason of variceal bleeding with preserved liver function due to non-cirrhotic portal hypertension.
系统性肥大细胞增多症是一种罕见的浸润性疾病,累及皮肤、骨髓、消化系统和肝脏。我们报告一例59岁男性病例,该患者出现大量静脉曲张出血,无任何肝硬化证据;然而,肝脏活检后来发现有严重的窦周纤维化伴门静脉区肥大细胞,骨髓有肥大细胞浸润且细胞增多。这个罕见病例描述了非肝硬化门静脉高压导致肝功能正常情况下静脉曲张出血的不同寻常原因。