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胆汁淤积性肝病的病理学诊断

张继平

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Citation:

胆汁淤积性肝病的病理学诊断

DOI: 10.12449/JCH240605
利益冲突声明:本文不存在任何利益冲突。
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    通信作者:

    张继平, zjp-zhang@163.com (ORCID: 0009-0000-4878-4607)

Pathological diagnosis of cholestatic liver disease

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    Corresponding author: ZHANG Jiping, zjp-zhang@163.com(ORCID: 0009-0000-4878-4607)
  • 摘要: 胆汁淤积性肝病(CLD)是指各种病因引起的胆汁代谢异常、流出受阻、胆管损伤等肝脏疾病,主要病因包括:药物、毒物、免疫、遗传、梗阻、感染、肿瘤等。胆汁淤积是CLD共有的病理改变,而不同病因淤胆的部位、组织病理及超微结构等改变,具有相对特异性。依据病因,重点阐述自身免疫性胆管炎、遗传代谢性肝病、大胆管病变的病理学特征,引申鉴别其他CLD,以期提高对CLD病理学的认识,助力精准诊疗。

     

  • 图  1  巨细胞变肝细胞(HE染色)

    注: a,×100;b,×200。较多巨细胞变肝细胞,胞体巨大,多核,胞质内可见淤胆性色素颗粒,汇管区小胆管损伤及轻微扩张,间质可见中性粒细胞等炎性细胞浸润。

    Figure  1.  Hepatocyte with giant-cell transformation(HE staining)

    图  2  PBC典型的病理学特征

    注: a,小胆管损伤伴淋巴细胞、浆细胞包围浸润及上皮样细胞肉芽肿,偶见多核巨细胞(HE染色,×100);b,旺炽型胆管炎,胆管上皮细胞嗜酸性化,核固缩,排列不整齐,管壁及管周较多淋巴细胞、浆细胞聚集浸润,散在少许嗜酸性粒细胞(HE染色,×200);c,CK7标记未见小胆管缺失,可见轻微细胆管反应(免疫组化,×100)。

    Figure  2.  Typical pathological features of PBC

    图  3  PSC典型的病理学特征

    注: a,肝硬化,汇管区轻度炎症,小胆管轻度扩张,管周纤维化(HE染色,×20);b,汇管区轻度炎症,小胆管基底膜增厚,周围“洋葱皮样”纤维化,小动脉扩大,管壁轻微增厚,门静脉分支扩张及管周纤维化,汇管区周围肝细胞气球样变性(HE染色,×100);c,免疫组化CK7显示肝细胞胆管化(IHC染色,×100);d,小胆管基底膜增厚(D-PAS染色,×100)。

    Figure  3.  Typical histopathological features of PSC

    图  4  肝豆状核变性的组织病理学及超微结构特征

    注: a,肝细胞水肿、脂肪变性、气球样变性,部分胞质呈淡红色颗粒状,腺泡1区少许糖原核肝细胞。汇管区轻度炎症(HE染色,×100);b,肝细胞胞质内较多红色铜颗粒沉积(罗丹宁铜染色,×100);c,肝细胞胞质内线粒体形态不规则,内外膜分类,可见内嵴扩张空泡及圆形高电子致密颗粒。肝细胞胞质内也可见脂滴、含高电子致密度的淤胆性色素颗粒沉积(TEM,×12 000)。

    Figure  4.  Histopathological and ultrastructural characteristics of WD

    图  5  CHF典型的组织病理学特征

    注: a,CHF“地图样”纤维化,胆管板畸形,胆汁淤积(HE染色,×40);b,汇管区胆管板畸形,门静脉狭窄、闭塞,肝细胞轻度水肿(HE染色,×100);c,畸形的胆管板内陷于汇管区纤维组织中,门静脉分支狭窄、闭塞(HE染色,×200);d,胆管板畸形,形态不规则,管腔扩张,可见胆栓,间质少许淋巴细胞、浆细胞浸润(HE染色,×200)。

    Figure  5.  Typical histopathological features of CHF

    图  6  EPP的组织病理学及超微结构特征

    注: a,肝细胞及毛细胆管内两种颜色的淤胆颗粒及胆栓,一种“巧克力”色,另一种淡黄色(HE染色,×100);b,偏振光显微镜可见红色双折光中的“Maltese十字”或星状暗区(HE染色,偏振光,×200);c,毛细胆管内可见原卟啉结晶(箭头),呈丝状弧形排列,毛细胆管腔面微绒毛减少,紧密连接延长(TEM,×15 000);d,Kupffer细胞胞质溶酶体内充满丝状原卟啉结晶(箭头),窦周间隙可见胶原纤维束沉积(TEM,×15 000)。

    Figure  6.  Histopathological and ultrastructural characteristics of EPP

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  • 收稿日期:  2024-04-02
  • 录用日期:  2024-05-14
  • 出版日期:  2024-06-25
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