| 王春迪丽努尔阿西木孙振柱胡玉斌.肺淋巴管平滑肌瘤病临床病理分析及文献复习[J].,2011,11(20):3868-3870 |
| 肺淋巴管平滑肌瘤病临床病理分析及文献复习 |
| Pulmonary Lmphangioleiomyomatosis :a Cinicpathological Aalysis andReview of Literature |
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| DOI： |
| 中文关键词: 淋巴管平滑肌瘤病 免疫组织化学 |
| 英文关键词: Pulmonary lymphangioleiomyomatosis Immunohistochemistry |
| 基金项目: |
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| 中文摘要: |
| 目的：探讨肺淋巴管平滑肌瘤病(PLAM)的临床和病理特征，提高对该病的认识。方法：对1 例PLAM 患者的临床特点、HE
及免疫组化染色结果进行分析，并结合文献进行复习。结果：PLAM 是一种持续发展的弥漫性肺疾病，几乎发生于育龄期妇女，主
要临床症状为进行性呼吸困难、反复气胸及乳糜胸。病理特征显示未成熟平滑肌样细胞在细支气管壁、肺泡壁、淋巴管壁和血管
壁周围增生。免疫组化染色显示增生PLAM 细胞Desmin、SMA、Vimentin、HMB45 均呈阳性表达, 部分细胞ER 及PR 阳性。结
论：PLAM 在影像学与病理组织学上有特征性表现，免疫组化HMB-45 阳性具特异性。由于肺部广泛病变，预后较差。对PLAM
的发病机制和治疗的研究已有了一定的进展，但今后尚需更深入地研究。 |
| 英文摘要: |
| Objective: To investigate the clinicopathological features of pulmonary lymphangioleiomyomatosis ( PLAM) and
enhance under standing. Methods: A PLAM case was analyzed by HE and immuno histochemical staining, and the related literature
reviewed. Results: PLAM was a rare diffuse interstitial plumonary disease and was almost restricted to females who were generally
premenopausal. The main clinicalmanifestations included dysnpea, pneumothorax and chylothorax. Pathological hallmarks of LAM are
diffuse infiltration of the pulmonary parenchyma with atypical smooth muscle cells, include bronchioles, lymphatics and blood vesssels .
IHC showed that characteristic smooth muscle cells were positive for Desmin, SMA, Vimentin, HMB45, estrogen and progesterone
receptor expression. Conclusions: PALM has characteristic manifestation of clinicopathology and radiology, IHC show positive of
HMB-45. As a diffuse lesion, the prognosis is worse. Now the research about the diagnosis and treatment of PLAM has improved, but we
must investigate it more deeply in the future. |
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