肺淋巴管平滑肌瘤病1例并文献复习
首发时间:2009-07-13
摘要:目的:探讨肺淋巴管平滑肌瘤病的临床特征,提高对本病的认识。方法:对1例肺淋巴管平滑肌瘤病患者的临床表现、影像学表现、病理结果、免疫组化等进行回顾性分析,并复习相关文献。结果:PLAM为一种原因不明的罕见病,主要见于育龄期女性,以呼吸困难、反复气胸、乳糜胸等为临床表现,胸部HRCT具有典型薄壁囊状改变,组织学以平滑肌细胞不典型增生为特点,HMB45染色具有重要意义。本病目前治疗困难,预后不佳。结论:临床医生因提高对本病的认识,及早诊断与治疗,探讨本病发病机制及寻找有效治疗措施是今后研究的重要课题。
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Clinical Analysis of Lymphangioleiomyomatosis
Abstract:This paper provides one case about a 41 years old woman with pulmonary Lymphangioleiomyomatosis, The clinical data of her were analyzed and corresponding literature was reviewed. Lymphangioleiomyomatosis (LAM) is a rare disease of unknown etiology, almost exclusively affecting women of child-bearing age. Its clinical characteristics include dyspnea, recurrent spontaneous pneumothorax, hemoptysis, chylothorax, and so on. High-resolution Computed tomography (HRCT) scans show numerous thin-walled cysts throughout the lungs. Microscopically, LAM consists of a diffuse proliferation of smooth muscle cells. Immunohistochemical stains specific for the HMB45-positive cell. No effective treatment currently exists for this progressive disorder. An increased awareness of LAM and its new diagnosis and therapeutic strategies can be made study to improve the prognosis.
Keywords: Lung tumor lymphangioleiomyomatosis smooth muscle proliferation
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No.3380325092212474****
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