Waldburger Nina, Rupp Christian, Klinke Sabine, Wieczorek Kathrin, Gotthardt Daniel, Kirchner Thomas, Schirmacher Peter, Straub Beate Katharina
Department of General Pathology, Institute of Pathology, Heidelberg University Hospital, Im Neuenheimer Feld 224, D-69120 Heidelberg, Germany.
Department of Internal Medicine IV, Heidelberg University Hospital, Im Neuenheimer Feld 410, D-69120 Heidelberg, Germany.
Hepat Oncol. 2015 Oct;2(4):343-347. doi: 10.2217/hep.15.33. Epub 2015 Nov 6.
Mastocytosis is a clonal, neoplastic mast cell proliferation, which is in most cases restricted to the skin (cutaneous mastocytosis), but may infiltrate other organs as well (systemic mastocytosis). Involvement of the liver by a systemic mastocytosis with impairment of liver function has been recognized as sign of an aggressive course of disease (C-finding). This article presents a case of aggressive systemic mastocytosis in a 26-year-old male patient with involvement of the liver mimicking primary sclerosing cholangitis. By histology we could demonstrate multifocal clusters of atypical mast cells infiltrating portal tracts in intimate contact with bile ducts as the cause of cholangitis and liver fibrosis.
肥大细胞增多症是一种克隆性肿瘤性肥大细胞增殖性疾病，大多数情况下局限于皮肤（皮肤肥大细胞增多症），但也可能浸润其他器官（系统性肥大细胞增多症）。系统性肥大细胞增多症累及肝脏并伴有肝功能损害已被认为是疾病侵袭性病程的标志（C级发现）。本文介绍了一例26岁男性侵袭性系统性肥大细胞增多症患者，其肝脏受累，酷似原发性硬化性胆管炎。通过组织学检查，我们发现多灶性非典型肥大细胞簇浸润门静脉区，与胆管紧密接触，这是胆管炎和肝纤维化的原因。
