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白消安肺

Busulphan lung.

作者信息

Littler W A, Kay J M, Hasleton P S, Heath D

出版信息

Thorax. 1969 Nov;24(6):639-55. doi: 10.1136/thx.24.6.639.

Abstract

A 61-year-old man with chronic myeloid leukaemia was treated with busulphan (Myleran). After receiving 1 g. of this drug over a period of 20 months he became dyspnoeic and developed crepitations in the lungs. Two months later radiographs of the chest revealed peri-hilar infiltrates and subsequently diffuse mottling throughout both lungs. Lung function tests showed a gross impairment of the transfer factor to a quarter of the predicted normal. At necropsy the lungs showed a striking proliferation of granular pneumocytes, many of which had disintegrated to produce intra-alveolar debris, some of which showed organization by fibrous tissue. There was associated interstitial pulmonary fibrosis. Electron microscopy confirmed the desquamated alveolar cells to be type II (granular) pneumocytes containing characteristic lamellar bodies. Many of these osmiophilic bodies, believed to be the source of pulmonary surfactant, had been liberated into the alveolar spaces, with the formation of phospholipid myelin figures and lattices. We think that the basic pathology of busulphan lung is a chemically induced alveolitis with proliferation of granular pneumocytes followed by fibrosis of alveolar walls and intra-alveolar contents.

摘要

一名61岁的慢性粒细胞白血病男性患者接受了白消安(马利兰)治疗。在20个月内接受了1克该药物后,他出现了呼吸困难并伴有肺部捻发音。两个月后,胸部X光片显示肺门周围浸润,随后两肺出现弥漫性斑点状阴影。肺功能测试显示转运因子严重受损,降至预测正常值的四分之一。尸检时,肺部显示颗粒性肺细胞显著增生,其中许多细胞已崩解产生肺泡内碎片,部分碎片显示有纤维组织机化。伴有间质性肺纤维化。电子显微镜证实脱落的肺泡细胞为Ⅱ型(颗粒性)肺细胞,含有特征性板层小体。许多这些嗜锇性小体被认为是肺表面活性物质的来源,已释放到肺泡腔内,形成磷脂髓鞘样结构和晶格。我们认为白消安肺的基本病理是化学诱导的肺泡炎,伴有颗粒性肺细胞增生,随后肺泡壁和肺泡内容物纤维化。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4d63/472068/a3681439b2cc/thorax00108-0002-a.jpg

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