Silvain C, Levillain P, Mouton P, Carretier M, Babin P, Beauchant M
Unité d'Hépatologie, CHU J.-Bernard, Poitiers.
Gastroenterol Clin Biol. 1989 Oct;13(10):834-7.
A 77-year-old woman had variceal bleeding related to systemic mastocytosis. Physical examination revealed minimal ascites and mild hepatomegaly noted 11 years before. Liver function tests were nearly normal. Because of early recurrent bleeding, a mesocaval shunt was performed. Wedged liver biopsy showed a moderate fibrosis of portal tracts and massive mast cell infiltration within portal tracts and sinusoids. Perisinusoidal collagen deposition was demonstrated ultrastructurally. We suggest that systemic mastocytosis be added to the list of diseases related portal hypertension with perisinusoidal fibrosis. As there is currently no specific treatment, a portocaval shunt should be discussed.
一名77岁女性因系统性肥大细胞增多症出现静脉曲张出血。体格检查发现有少量腹水，11年前曾发现轻度肝肿大。肝功能检查基本正常。由于早期复发出血，进行了肠系膜上腔静脉分流术。楔形肝活检显示门管区中度纤维化，门管区和肝血窦内有大量肥大细胞浸润。超微结构显示肝血窦周围有胶原沉积。我们建议将系统性肥大细胞增多症列入与肝血窦周围纤维化相关的门静脉高压疾病名单。由于目前尚无特效治疗方法，应考虑进行门腔静脉分流术。
