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伴有系统性浆细胞增多症的肝炎相关性再生障碍性贫血。

Hepatitis-associated aplastic anemia with systemic plasmacytosis.

作者信息

Nishimoto Y, Iwahashi T, Nishihara T, Katayama H, Kuribayashi K, Takao T, Saito K

出版信息

Acta Pathol Jpn. 1987 Jan;37(1):155-66. doi: 10.1111/j.1440-1827.1987.tb03143.x.

Abstract

An autopsy case of hepatitis associated aplastic anemia was presented. A 58-year-old Japanese female with non-A, non-B hepatitis was admitted on August 2, 1983. Moderate grade of fever and hemorrhagic diathesis appeared on September 16, when hepatitis was evaluated as being under resolving. The peripheral blood and bone marrow findings were consistent with aplastic anemia. Since infection was suggested by increased levels of serum gammaglobulin and CRP, treatment with antibiotics as well as prednisolone and blood transfusion was initiated. Since September 21, gradual tenderness and edema on the right lower abdominal wall appeared. She died on October 3. On postmortem examination, systemic plasmacytosis with lymphadenopathy and septic monilial infection was revealed. Numerous plasma cells were atypical, but were immunohistochemically proved to be polyclonal. The bone marrow showed a massive and diffuse plasma cell proliferation with extremely scarce myeloid cells and megakaryocytes. There was a large granulomatous lesion with monilial infection in the wall of the ileocecum. By these findings, systemic plasmacytosis was suspected to be due to chronic monilial infection. The pathogenesis of systemic plasmacytosis in aplastic anemias and in other diseases were discussed with relation to the present case.

摘要

本文报告1例肝炎相关性再生障碍性贫血的尸检病例。一名58岁的日本女性,患有非甲非乙型肝炎,于1983年8月2日入院。9月16日,当评估肝炎正在好转时,出现中度发热和出血倾向。外周血和骨髓检查结果符合再生障碍性贫血。由于血清γ球蛋白和CRP水平升高提示有感染,遂开始使用抗生素、泼尼松龙治疗及输血。9月21日起,右下腹壁逐渐出现压痛和水肿。患者于10月3日死亡。尸检发现全身浆细胞增多伴淋巴结病和念珠菌败血症感染。大量浆细胞为非典型性,但免疫组化证实为多克隆性。骨髓显示大量弥漫性浆细胞增生,髓系细胞和巨核细胞极度稀少。回盲部肠壁有一个伴有念珠菌感染的大肉芽肿性病变。根据这些发现,怀疑全身浆细胞增多是由慢性念珠菌感染所致。结合本病例,对再生障碍性贫血及其他疾病中全身浆细胞增多的发病机制进行了讨论。

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