Lu Lan, Wang Zhijie, Li Huawei, Li Xue, Ma Shuangyue, Wang Luhua, Yang Boyan
Department of Comprehensive Oncology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital & Shenzhen Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Shenzhen, China.
Department of Medical Oncology, State Key Laboratory of Molecular Oncology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Ann Palliat Med. 2021 Jan;10(1):767-777. doi: 10.21037/apm-20-2220. Epub 2021 Jan 15.
Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare but severe pulmonary complication of malignant tumors. It is characterized by the presence of multiple microscopic tumor emboli attached to the endothelium of small pulmonary arteries and induces fibrointimal proliferation and the activation of coagulation. The main clinical manifestations of PTTM are dyspnea, dry cough, hypoxemia, pulmonary hypertension, right heart failure, thrombocytopenia, and disseminated intravascular coagulation (DIC). Chest computed tomography (CT) shows no distinctive findings, and PTTM is often unrecognized and universally underdiagnosed, with an appalling prognosis. An antemortem diagnosis of PTTM is also difficult due to a lack of specific clinical and imaging features. Moreover, there is presently no therapeutic management and the illness rapidly progresses to death. Early identification and timely and effective use of oncotherapy can help to alleviate symptoms and improve prognosis. According to recent reports, targeting angiogenesis factors including platelet-derived growth factor (PDGF) and vascular endothelial growth factor (VEGF) have marginally improved survival. In this article, we report the case of a patient with advanced lung adenocarcinoma complicated with PTTM and DIC. A combined therapeutic regimen of bevacizumab with pemetrexed successfully improved chest CT findings, respiratory symptoms, DIC, and short-term outcomes. Anti-angiogenesis drugs modify the pulmonary vascular structure, and rapidly improve the lung tumor microenvironment. And therefore, it may be a potentially effective drug for the treatment of PTTM.
肺肿瘤血栓性微血管病(PTTM)是一种罕见但严重的恶性肿瘤肺部并发症。其特征是多条微小肿瘤栓子附着于肺小动脉内皮,可导致纤维内膜增生和凝血激活。PTTM的主要临床表现为呼吸困难、干咳、低氧血症、肺动脉高压、右心衰竭、血小板减少和弥散性血管内凝血(DIC)。胸部计算机断层扫描(CT)无特异性表现,PTTM常未被认识且普遍诊断不足,预后极差。由于缺乏特异性的临床和影像学特征,PTTM的生前诊断也很困难。此外,目前尚无有效的治疗方法,病情迅速进展至死亡。早期识别并及时有效地使用肿瘤治疗方法有助于缓解症状并改善预后。根据最近的报道,靶向包括血小板衍生生长因子(PDGF)和血管内皮生长因子(VEGF)在内的血管生成因子可使生存率略有提高。在本文中,我们报告了1例晚期肺腺癌合并PTTM和DIC患者的病例。贝伐单抗联合培美曲塞的联合治疗方案成功改善了胸部CT表现、呼吸道症状、DIC及短期预后。抗血管生成药物可改变肺血管结构,并迅速改善肺肿瘤微环境。因此,它可能是治疗PTTM的一种潜在有效药物。