Story Maria, Kwon Sook Kyung, Robinson Robert, Fortis Spyridon
Department of Internal Medicine, University of Iowa Hospitals and Clinics, Iowa City, Iowa, USA.
Department of Pathology, University of Iowa Hospitals and Clinics, Iowa City, Iowa, USA.
BMJ Case Rep. 2017 Jun 28;2017:bcr-2017-219730. doi: 10.1136/bcr-2017-219730.
We report the case of a previously healthy man who presented with subacute dyspnoea after a long drive. He developed hypoxic respiratory failure, thought secondary to a massive pulmonary embolism and was treated with tissue plasminogen activator but died in the hospital despite aggressive medical measures. Autopsy revealed pulmonary tumour thrombotic microangiopathy (PTTM) from papillary renal cell carcinoma. PTTM is a rare clinicopathological syndrome that clinically results in symptoms of dyspnoea and right heart failure. Pathologically, a localised paraneoplastic process evolves from tumour microemboli in the pulmonary arterioles, resulting in fibrocellular proliferation and narrowing of the vessels, causing subacute right heart failure. To our knowledge, this is the first case of PTTM due to papillary renal cell carcinoma.
我们报告一例既往健康的男性病例，该患者在长途驾车后出现亚急性呼吸困难。他发展为低氧性呼吸衰竭，考虑继发于大面积肺栓塞，接受了组织型纤溶酶原激活剂治疗，但尽管采取了积极的医疗措施，仍在医院死亡。尸检显示为乳头状肾细胞癌导致的肺肿瘤血栓性微血管病（PTTM）。PTTM是一种罕见的临床病理综合征，临床上表现为呼吸困难和右心衰竭症状。病理上，一种局限性的副肿瘤过程由肺小动脉中的肿瘤微栓子演变而来，导致纤维细胞增生和血管狭窄，引起亚急性右心衰竭。据我们所知，这是首例由乳头状肾细胞癌导致的PTTM病例。
