伴有肺部表现的α链病

Florin-Christensen A, Doniach D, Newcomb P B

Br Med J. 1974 May 25;2(5916):413-5. doi: 10.1136/bmj.2.5916.413.

A case of alpha-chain disease of the pulmonary type is described in a man presenting with dyspnoea, mottling on chest x-ray picture, and a CO-transfer factor of 36%, suggestive of fibrosing alveolitis. The serum IgA consisted entirely of abnormal alpha chains devoid of light chains. This protein had the unusual property of reacting by immunofluorescence with rat mitochondria but not with human issues or with those of other species. Postmortem examination showed enlarged mediastinal lymph nodes with no evidence of malignancy or fibrosing alveolitis.

本文描述了一例肺型α链病，患者为一名男性，表现为呼吸困难、胸部X光片出现斑点状阴影，一氧化碳转运因子为36%，提示为纤维化肺泡炎。血清IgA完全由不含轻链的异常α链组成。这种蛋白质具有一种不寻常的特性，即通过免疫荧光与大鼠线粒体发生反应，但不与人或其他物种的组织发生反应。尸检显示纵隔淋巴结肿大，无恶性肿瘤或纤维化肺泡炎的证据。