Department of Respiratory Medicine, Toho University Omori Medical Center, Omorinishi 6-11-1, Ota-ku, Tokyo 143-8541, Japan.
Diagn Pathol. 2013 Aug 6;8:134. doi: 10.1186/1746-1596-8-134.
This study presents an extremely rare case of constrictive bronchiolitis obliterans (BO) associated with Stevens-Johnson Syndrome (SJS) provides the morphological and immunohistochemical features using histopathological bronchial reconstruction technique. A 27-year-old female developed progressive dyspnea after SJS induced by taking amoxicillin at the age of 10. Finally, she died of exacerbation of type II respiratory failure after 17 years from clinically diagnosed as having BO. Macroscopic bronchial reconstruction of the whole lungs at autopsy showed the beginning of bronchial obliterations was in the 4th to 5th branches, numbering from each segmental bronchus. Once they were obliterated, the distal and proximal bronchi were dilated. Microscopic bronchial reconstruction demonstrated the localization of obliteration was mainly from small bronchi to membranous bronchioli with intermittent airway luminal narrowing or obliteration. Moreover, CD3-, CD20-, and CD68-positive cells were found in the BO lesions. CD34- and D2-40-positive cells were mainly distributed in the peribronchiolar lesions and bronchiolar lumens, respectively. SMA- and TGF-β-positive cells were seen in the fibrous tissue of BO lesions.
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本研究报告了一例极为罕见的闭塞性细支气管炎(BO)伴史蒂文斯-约翰逊综合征(SJS)的病例,采用支气管重建技术提供了形态学和免疫组织化学特征。一名 27 岁女性,10 岁时因服用阿莫西林引起 SJS 后出现进行性呼吸困难,17 年后因 II 型呼吸衰竭加重而死亡,最终临床诊断为 BO。尸检时全肺支气管宏观重建显示支气管闭塞始于第 4 至 5 级分支,从每个节段性支气管开始。一旦发生闭塞,远端和近端支气管就会扩张。支气管微观重建显示闭塞的定位主要从小支气管到膜性细支气管,伴有间歇性气道管腔狭窄或闭塞。此外,BO 病变中发现 CD3-、CD20-和 CD68 阳性细胞。CD34-和 D2-40 阳性细胞主要分布在支气管周围病变和细支气管腔中,分别为 SMA-和 TGF-β阳性细胞见于 BO 病变的纤维组织中。
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