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Case of autoimmune hepatitis with markedly enlarged hepatoduodenal ligament lymph nodes

机译:自身免疫性肝炎伴肝十二指肠韧带淋巴结明显肿大的病例

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Autoimmune hepatitis (AIH) is a necroinflammatory liver disease of unknown etiology. The disease is characterized histologically by interface hepatitis, biochemically by increased a spartate amino transferase and alanine aminotransferase levels, and serologically by increased autoantibodies and immunoglobulin G levels. Here we discuss AIH in a previously healthy 37-year-old male with highly elevated serum levels of soluble inter-leukin-2 receptor and markedly enlarged hepatoduodenal ligament lymph nodes (HLLNs, diameter, 50 mm). Based on these observations, the differential diagnoses were AIH, lymphoma, or Castleman's disease. Liver biopsy revealed the features of interface hepatitis without bridging fibrosis along with plasma cell infiltration which is the typical characteristics of acute AIH. Lymph node biopsy revealed lymphoid follicles with inflammatory lymphocytic infiltration; immunohistochemical examination excluded the presence of lymphoma cells. Thereafter, he was administered corticosteroid therapy: after 2 mo, the enlarged liver reached an almost normal size and the enlarged HLLNs reduced in size. We could not find AIH cases with such enlarged lymph nodes (diameter, 50 mm) in our literature review. Hence, we speculate that markedly enlarged lymph nodes observed in our patient may be caused by a highly activated, humoral immune response in AIH.
机译:自身免疫性肝炎(AIH)是一种病因不明的坏死性炎症性肝病。该疾病在组织学上以界面性肝炎为特征,在生化上以谷草转氨酶和丙氨酸氨基转移酶水平升高为特征,在血清学上以自身抗体和免疫球蛋白G水平升高为特征。在这里,我们讨论了一个先前健康的37岁男性的AIH,其血清可溶性白介素2受体的血清水平高度升高,肝十二指肠韧带淋巴结明显肿大(HLLN,直径50 mm)。根据这些观察结果,鉴别诊断为AIH,淋巴瘤或Castleman病。肝活检显示界面肝炎的特征是没有桥接纤维化以及浆细胞浸润,这是急性AIH的典型特征。淋巴结活检显示淋巴滤泡有炎性淋巴细胞浸润。免疫组织化学检查排除了淋巴瘤细胞的存在。此后,他接受了皮质类固醇激素疗法:2个月后,肝脏肿大达到了几乎正常的大小,而肿大的HLLN缩小了。在我们的文献综述中,我们没有发现淋巴结肿大(直径50 mm)的AIH病例。因此,我们推测在我们的患者中观察到的淋巴结明显增大可能是由于AIH中高度激活的体液免疫反应所致。

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