Critical Care Medicine Department, National Institutes of Health Clinical Center, Bethesda, MD, USA.
Women's Malignancies Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, USA.
BMC Pulm Med. 2018 Jul 11;18(1):112. doi: 10.1186/s12890-018-0681-x.
Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare cause of pulmonary hypertension that is associated with malignancies and is marked by the presence of non-occlusive tumor emboli and fibrocellular intimal proliferation of small pulmonary arteries leading to increased pulmonary vascular resistance and right heart failure. The diagnosis of PTTM is challenging to make pre-mortem and guidelines on treatment are lacking.
A 45-year-old woman with advanced squamous cell carcinoma of the cervix developed symptoms of dyspnea and evidence of right heart failure during a phase I clinical trial with cediranib and durvalumab. After an extensive evaluation, pre-capillary pulmonary hypertension was confirmed by right heart catheterization. Vasodilator therapy was initiated but resulted in the development of symptomatic hypoxemia and was discontinued. Despite continued supportive care, she continued to decline and was transitioned to hospice care. At autopsy, the cause of her right heart failure was found to be due to PTTM with features of pulmonary veno-occlusive disease (PVOD).
PTTM and PVOD are important diagnoses to consider in patients with a malignancy and the development of right heart failure and may be manifestations of a spectrum of similar disease processes.
肺肿瘤血栓性微血管病（PTTM）是一种罕见的肺动脉高压病因，与恶性肿瘤相关，其特征为非闭塞性肿瘤栓子和小肺动脉纤维细胞内膜增殖，导致肺血管阻力增加和右心衰竭。PTTM 的生前诊断具有挑战性，且缺乏治疗指南。
一名 45 岁女性患有晚期宫颈鳞状细胞癌，在 cediranib 和 durvalumab 的 I 期临床试验中出现呼吸困难症状和右心衰竭证据。经过广泛评估，通过右心导管检查确认存在毛细血管前肺动脉高压。开始使用血管扩张剂治疗，但导致症状性低氧血症，并停止使用。尽管持续进行支持性护理，她的病情仍持续恶化，并转入临终关怀。尸检发现，她的右心衰竭是由 PTTM 引起的，伴有肺静脉闭塞性疾病（PVOD）的特征。
在患有恶性肿瘤和右心衰竭的患者中，PTTM 和 PVOD 是需要考虑的重要诊断，可能是一系列类似疾病过程的表现。
