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自身免疫性肝炎的病理学诊断

杨文君

引用本文:
Citation:

自身免疫性肝炎的病理学诊断

DOI: 10.12449/JCH240603
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:

    杨文君, yangwenjun3463@163.com (ORCID: 0000-0003-2021-595X)

Pathological diagnosis of autoimmune hepatitis

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  • 摘要:

    自身免疫性肝炎(AIH)是免疫介导的肝实质炎症性疾病,以高丙种球蛋白血症、自身抗体阳性和典型的肝组织学异常为特征。然而,AIH临床表现多样,缺乏特异性的血清学标志物,因此AIH的诊断困难,具有挑战性。虽然汇管区淋巴浆细胞浸润、界面性肝炎、淋巴细胞穿入现象和肝细胞玫瑰花结为AIH典型的组织学特征,但在AIH中尚可观察到其他的一些形态学改变,包括小叶中心性坏死、Kupffer细胞内透明小球等。所以没有一个单一的组织学特征可直接诊断AIH,需结合临床和实验室检查,并排除其他原因引起的肝脏疾病,方可做出正确的诊断。本文总结了AIH的组织学特征、不同的病理组织学谱、常见的临床问题、主要的鉴别诊断和最新进展。

     

  • 图  1  汇管区淋巴浆细胞浸润和界面性肝炎

    注: a,汇管区淋巴浆细胞浸润,界面性肝炎(HE染色,×10)b,汇管区簇状浆细胞浸润(箭头)(HE染色,×40);c,簇状浆细胞阳性(箭头)(MUM-1染色,×20);d,簇状浆细胞阳性(箭头)(CD38染色,×20)。

    Figure  1.  Portal lymphoplasmacytic infiltrate and interface hepatitis

    图  2  淋巴细胞穿入(HE染色,×40)

    注: 箭头处为淋巴细胞穿入。

    Figure  2.  Emperipolesis(HE staining,×40)

    图  3  肝细胞玫瑰花结(HE染色,×20)

    注: 箭头处为肝细胞玫瑰花结。

    Figure  3.  Hepatocyte rosettes(HE staining,×20)

    图  4  小叶中心性坏死(HE染色,×10)

    注: 箭头处为小叶中心性坏死。

    Figure  4.  Centrilobular necrosis(HE staining,×10)

    图  5  合胞体多核巨肝细胞(HE染色,×20)

    注: 箭头处为合胞体多核巨肝细胞。

    Figure  5.  Syncytial multinucleated giant cells(HE staining,×20)

    表  1  基于汇管区和小叶炎症的AIH诊断标准

    Table  1.   AIH diagnostic criteria based on portal area and lobular inflammation

    诊断 汇管区炎症 小叶炎症
    极可能 (likely AIH) 汇管区淋巴、浆细胞浸润+ 以下1种或2种特征: (1)轻度以上界面性肝炎, (2)轻度以上小叶炎; 缺乏提示其他肝病的组织学形态 轻度以上小叶炎(伴或不伴小叶中心性坏死)+至少1种以下特征: (1)淋巴、浆细胞浸润, (2)界面性肝炎, (3)汇管区纤维化; 缺乏提示其他肝病的组织学形态
    可能 (possible AIH) 汇管区淋巴、浆细胞浸润; 不具有上述极可能的任一特征; 缺乏提示其他肝病的组织学形态或 伴上述1种或2种极可能的特征; 出现提示其他肝病的组织学形态 小叶性肝炎,伴或不伴小叶中心性坏死; 不具有上述极可能的任一特征; 缺乏提示其他肝病的组织学形态或 伴上述任一极可能的特征; 出现提示其他肝病的组织学形态
    不太可能 (likely AIH) 汇管区炎症; 不具有上述极可能的任一特征; 出现提示其他肝病的组织学形态 任何程度的小叶性肝炎; 不具有上述极可能的任一特征; 出现提示其他肝病的组织学形态
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  • 收稿日期:  2024-03-30
  • 录用日期:  2024-05-15
  • 出版日期:  2024-06-25
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