Godbole Rohit H, Saggar Rajan, Kamangar Nader
1 Division of Pulmonary and Critical Care Medicine, University of California, Irvine, CA, USA.
2 Division of Pulmonary and Critical Care Medicine, University of California, Los Angeles David Geffen School of medicine, Los Angeles, CA, USA.
Pulm Circ. 2019 Apr-Jun;9(2):2045894019851000. doi: 10.1177/2045894019851000.
Pulmonary tumor thrombotic microangiopathy (PTTM) is a fatal disease process in which pulmonary hypertension (PH) develops in the setting of malignancy. The purpose of this study is to present a detailed analysis of cases of PTTM reported in literature in the hopes of achieving more ante-mortem diagnoses. We conducted a systematic review of currently published and available cases of PTTM by searching the term "pulmonary tumor thrombotic microangiopathy" on the Pubmed.gov database. Seventy-nine publications were included consisting of 160 unique cases of PTTM. The most commonly reported malignancy was gastric adenocarcinoma (94 cases, 59%). Cough and dyspnea were reported in 61 (85%) and 102 (94%) cases, respectively. Hypoxemia was reported in 96 cases (95%). Elevation in D-dimer was noted in 36 cases (95%), presence of anemia in 32 cases (84%), and thrombocytopenia in 30 cases (77%). Common findings on chest computed tomography (CT) included ground-glass opacities (GGO) in 28 cases (82%) and nodules in 24 cases (86%). PH on echocardiography was noted in 59 cases (89%) with an average right ventricular systolic pressure of 71 mmHg. Common features of PTTM that are reported across the published literature include presence of dyspnea and cough, hypoxemia, with abnormal CT findings of GGO, nodules, and mediastinal/hilar lymphadenopathy, and PH. PTTM is a universally fatal disease process and this analysis provides a detailed examination of all the available published data that may help clinicians establish an earlier diagnosis of PTTM.
肺肿瘤血栓性微血管病（PTTM）是一种致命的疾病过程，在恶性肿瘤背景下会发生肺动脉高压（PH）。本研究的目的是对文献中报道的PTTM病例进行详细分析，以期实现更多的生前诊断。我们通过在Pubmed.gov数据库中搜索“肺肿瘤血栓性微血管病”一词，对目前已发表的PTTM病例进行了系统评价。纳入了79篇出版物，其中包含160例独特的PTTM病例。最常报道的恶性肿瘤是胃腺癌（94例，59%）。分别有61例（85%）和102例（94%）报告有咳嗽和呼吸困难。96例（95%）报告有低氧血症。36例（95%）D-二聚体升高，32例（84%）有贫血，30例（77%）有血小板减少。胸部计算机断层扫描（CT）的常见表现包括28例（82%）磨玻璃影（GGO）和24例（86%）结节。超声心动图显示59例（89%）有PH，平均右心室收缩压为71 mmHg。已发表文献中报道的PTTM的常见特征包括呼吸困难、咳嗽、低氧血症，CT表现异常，如GGO、结节以及纵隔/肺门淋巴结肿大，还有PH。PTTM是一种普遍致命的疾病过程，本分析对所有可用的已发表数据进行了详细审查，可能有助于临床医生更早地诊断PTTM。
