肺淋巴瘤样肉芽肿病3例报告并文献复习

Pulmonary Lymphomatoid Granulomatosis:Three Cases Report and Literature Review

目的:探讨肺淋巴瘤样肉芽肿病(LyG)的临床特点,诊断要素,提高对LyG的认识。方法:总结我院收治的经开胸或经皮穿刺肺活检确诊的3例LyG患者的临床资料,分析其临床、病理特点和诊疗依据。结果:LyG为多系统疾病,主要累及肺部,其次是皮肤和中枢神经系统。X线是发现本病的主要手段,病理是确诊的主要依据。部分患者经化、放疗病情缓解。结论:LyG是一种与免疫缺陷和EB病毒感染有关的富含T细胞的B细胞淋巴瘤。临床少见,易误诊。组织学上具有多形性淋巴样细胞浸润、血管炎及肉芽肿三联征。总的预后差,Ⅲ级的患者需联合化疗。

Objective：To discuss the clinical characteristic and the diagnostic keys of pulmonary lymphomatoid granulomatosis（LyG）. Method：We reviewed the clinical materials of the 3 hospitalized patients with LyG diagnosed by pulmonary biopsy through thoracocentesis or thoracic surgery, analyzed the results of the clinical manifestation, pathology, diagnosis and the therapy. Results：LyG was a kind of the polysystemic disease, which involved in the lung and skin. X-ray was the main method to detect the disease. Pathology was the key method to the diagnosis. Chemotherapy and radiotherapy were effective. Conclusion ：LyG is a T-cell rich B-cell lymphoma which is associated with the immunodeficiency and the infection of EB virus ;It is rare in the clinic and is very easily misdiagnosed. The histologic trigeminy of the LyG pathologic manifestation involves the infiltration of the polymorphic lymphomatoid cells, angitis and granulomatosis. The prognosis is poor. Ⅲ grade patients need combine with chemotherapy.