弥漫性肺淋巴管瘤病1例报道及文献复习  

Diffuse pulmonary lymphangiomatosis : clinical and pathologic analysis and review of literature

作　　者：闫利娟 易祥华[2] 朱旭友[2] 顾盼[2] 曾郁[2] 吴运瑾[2] 张素霞[2] 

机构地区：[1]洛阳市第二中医院病理科,河南洛阳471003 [2]同济大学附属同济医院病理科,上海200065

出　　处：《诊断学理论与实践》2013年第2期170-174,共5页Journal of Diagnostics Concepts & Practice

基　　金：上海市科学技术委员会科研基金资助项目(034119868);上海市科学技术委员会医学重点科研基金资助项目(09411951600)

摘　　要：目的:探讨弥漫性肺淋巴管瘤病(diffuse pulmonary lymphangiomatosis,DPL)患者的临床病理特征,以提高对该病的诊断水平。方法:观察1例DPL患者的的临床表现、影像学特点,分析其开胸肺活检病理形态特征及免疫组织化学表型,并结合文献进行探讨。结果:患者为青年男性,主要临床表现为咳嗽、咯血和乳糜胸;胸部CT显示双肺多发结节影、网格影;病理示肺组织内淋巴管增生和扩张,免疫组化显示淋巴管内皮细胞CD31和D2-40阳性染色。结论:DPL患者的临床及影像学表现均缺乏特征性,双肺多发结节状和网格状阴影伴乳糜胸是其主要特点,明确诊断依赖于肺活检病理诊断。Objective To investigate the clinicopathologic characteristics of diffuse pulmonary lymphangiomatosis （DPL） for improving the diagnosis. Methods One case of DPL was observed for its clinical symptoms, imaging features, open-lung biopsy pathological characteristics and immunohistochemical phenotypes, also relevant literatures were reviewed. Results The main clinical manifestation of this young male patient was characterized by cough, hemoptysis and chylothorax. Chest CT showed multiple lung nodules and grid-like shadow. Pathological examination showed lymphatic vessel hyperplasia and expansion in lung tissue. And immunohistochemistry showed positive staining of lymphatic endothelial cells CD31 and D2-40. Conclusions The clinical and imaging appearances of DPL are lack of characterization, and its main characteristics are multiple lung nodules and grid-like shadow with chylothorax. Its diagnosis unequivocally depends on the lung biopsy pathology.

关 键 词：弥漫性肺淋巴管瘤病 诊断 鉴别诊断 

分 类 号：R563.13[医药卫生—呼吸系统]