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伴有蛋白异常血症的血管免疫母细胞性淋巴结病中的间质性肺炎。一份伴有特殊组织病理学研究的病例报告。

Interstitial pneumonia in angio-immunoblastic lymphadenopathy with dysproteinemia. A case report with special histopathologic studies.

作者信息

Iseman M D, Schwarz M I, Stanford R E

出版信息

Ann Intern Med. 1976 Dec;85(6):752-5. doi: 10.7326/0003-4819-85-6-752.

Abstract

A patient presented with characteristic historical, physical, and laboratory findings of angio-immunoblastic lymphadenopathy with dysproteinemia. This newly described entity apparently represents a nonneoplastic proliferation of the B-lymphocyte system with immunoblastic transformation of many lymphocytes and excessive production of immunoglobulins. It is associated with fever, sweats, weight loss, skin rash, lymphadenopathy, splenomegaly, hepatomegaly, and characteristic histologic features of the involved lymph nodes. Noteworthy in the patient reported here are the extent and course of radiographically and clinically evident pulmonary involvement and the biopsy documentation of an interstitial pneumonia marked by histopathologic changes closely resembling those found in the lymph nodes, with immunohistologic demonstration of immunoglobulins in the alveolar walls.

摘要

一名患者出现了血管免疫母细胞性淋巴结病伴蛋白异常血症的典型病史、体格检查及实验室检查结果。这个新描述的实体显然代表了B淋巴细胞系统的非肿瘤性增殖,伴有许多淋巴细胞的免疫母细胞转化及免疫球蛋白的过度产生。它与发热、盗汗、体重减轻、皮疹、淋巴结病、脾肿大、肝肿大以及受累淋巴结的特征性组织学特征相关。在此报道的患者中值得注意的是影像学和临床上明显的肺部受累的范围及病程,以及活检证实的间质性肺炎,其组织病理学改变与在淋巴结中发现的改变极为相似,免疫组织学显示肺泡壁中有免疫球蛋白。

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