Association of primary sclerosing cholangitis, thymoma and hypogammaglobulinemia.

R Yoshioka, Y Sato, A Kogure, H Ohira, T Takagi, M Kuroda, M Miyata, K Obara, T Nishimaki, R Kasukawa
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引用次数: 11

Abstract

A 64-year-old Japanese woman with thymoma has been suffering from diarrhea and increased alkaline phosphatase levels without jaundice. Her serum immunoglobulin levels of IgM and IgG were less than half of the normal levels, with an increase in CD8 (suppressor/cytotoxic) T cell percentage and a decrease in CD4 (helper) T cell percentage, resulting in a lower CD4/CD8 ratio of 0.31. These immunological features are in accordance with those of hypogammaglobulinemia complicating thymoma. Cholangiography and a liver biopsy specimen disclosed the presence of primary sclerosing cholangitis (PSC). PSC has been recognized in various immunodeficiency syndromes and this case shows that thymoma complicated by hypogammaglobulinemia is associated with PSC.

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原发性硬化性胆管炎、胸腺瘤和低丙种球蛋白血症的关系。
一名64岁的日本妇女患有胸腺瘤,一直患有腹泻和碱性磷酸酶水平升高,但没有黄疸。血清IgM和IgG免疫球蛋白水平低于正常水平的一半,CD8(抑制性/细胞毒性)T细胞百分比升高,CD4(辅助性)T细胞百分比降低,CD4/CD8比值降低至0.31。这些免疫学特征与低γ -球蛋白血症合并胸腺瘤的免疫学特征一致。胆管造影和肝活检标本显示原发性硬化性胆管炎(PSC)的存在。PSC已被确认在多种免疫缺陷综合征中,本病例显示胸腺瘤合并低γ球蛋白血症与PSC有关。
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