Yu Min, Huang Weibin, Wang Yan, Wang Guangsuo, Wang Lingwei, Tao Weihua, Faiz Saadia A, Ng Fung Him, Li Huiping
Department of Respiratory and Critical Care Medicine, Shenzhen People's Hospital & the First Affiliated Hospital of Southern University of Science and Technology & the Second Clinical School of Jinan University & Shenzhen Municipal Institute of Respiratory Medicine, Shenzhen, China.
Department of Pathology, Shenzhen People's Hospital & the First Affiliated Hospital of Southern University of Science and Technology & the Second Clinical School of Jinan University & Shenzhen Municipal Institute of Respiratory Medicine, Shenzhen, China.
Ann Transl Med. 2021 Jan;9(1):74. doi: 10.21037/atm-20-7441.
This study aimed to analyze the diagnosis and treatment of one case of pulmonary angiosarcoma (PPA) retrospectively. The main manifestation of this female patient was cough, hemoptysis and dyspnea. Computed tomography (CT) of the chest revealed multiple small nodules and ground-glass patches in both lungs suggesting of diffuse alveolar hemorrhage (DAH). Laboratory examination revealed decreased hemoglobin and platelet counting, normal coagulation function. Results of rheumatic markers testing including antinuclear antibody (ANA), anti-extractable nuclear antigen antibody (ENA), vasculitis marker, and antiphospholipid antibody were negative. Tumor markers were negative. Sputum smear, sputum culture, and alveolar lavage fluid culture showed negative results. The bone marrow smear was essentially normal. The patient received methylprednisolone pulse therapy (250 mg daily × 5 days) and immunoglobin (20 d daily × 7 days) treatment, but her hemoptysis persisted. Bilateral pleural effusion drainage found a large amount of bloody effusion, but cytology of the pleural fluid showed negative results. The clinical symptoms, laboratory results, imaging findings, and pathological features of the patient were summarized, and problems in diagnosis and treatment were discussed. A thoracoscopic lung biopsy was performed and the diagnosis of PPA was confirmed by pathology and immunohistochemistry (IHC) staining. This case suggested that the possibility of PPA should be considered in patients with DAH, but with negative findings in routine examinations, lung biopsy is usually required.
本研究旨在回顾性分析1例肺血管肉瘤（PPA）的诊断与治疗情况。该女性患者的主要表现为咳嗽、咯血和呼吸困难。胸部计算机断层扫描（CT）显示双肺有多个小结节和磨玻璃影，提示弥漫性肺泡出血（DAH）。实验室检查显示血红蛋白和血小板计数降低，凝血功能正常。包括抗核抗体（ANA）、抗可提取核抗原抗体（ENA）、血管炎标志物和抗磷脂抗体在内的风湿标志物检测结果均为阴性。肿瘤标志物为阴性。痰涂片、痰培养及肺泡灌洗液培养结果均为阴性。骨髓涂片基本正常。患者接受了甲泼尼龙冲击治疗（每日250mg×5天）和免疫球蛋白治疗（每日20g×7天），但咯血仍持续存在。双侧胸腔积液引流发现大量血性胸水，但胸水细胞学检查结果为阴性。总结了该患者的临床症状、实验室检查结果、影像学表现及病理特征，并讨论了诊断和治疗中存在的问题。进行了胸腔镜肺活检，病理及免疫组织化学（IHC）染色确诊为PPA。该病例提示，对于DAH患者，若常规检查结果为阴性，应考虑PPA的可能性，通常需要进行肺活检。
