Hutchinson Josh Curtis, Fulcher James W, Hanna Jeff, Ward Michael E
From the *Department of Public Health Sciences, Clemson University, Clemson; †Office of the Medical Examiner, Pathology Associates of Greenville and the Greenville Health System University Medical Center; and ‡Department of Radiology, Greenville Health System, Greenville County, SC.
Am J Forensic Med Pathol. 2018 Mar;39(1):56-60. doi: 10.1097/PAF.0000000000000369.
Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare rapidly progressive fatal disease that is difficult to diagnosis antemortem. Activation of the coagulation cascade and fibrocellular intimal thickening caused by embolism of tumor cells into pulmonary vasculature leads to extensive pulmonary hypertension and eventually death. We describe a case of PTTM with association of a presumed lung adenocarcinoma primary. Although rare in nature, PTTM should be a considered diagnosis with chronic dyspnea of unknown origin, severe pulmonary hypertension coupled with right-side heart failure, in the absence of pulmonary embolism.
肺肿瘤血栓性微血管病(PTTM)是一种罕见的、进展迅速的致命疾病,生前难以诊断。肿瘤细胞栓塞至肺血管系统导致凝血级联反应激活和纤维细胞内膜增厚,进而引发广泛的肺动脉高压并最终导致死亡。我们描述了一例与疑似原发性肺腺癌相关的PTTM病例。尽管PTTM本质上较为罕见,但对于不明原因的慢性呼吸困难、严重肺动脉高压合并右侧心力衰竭且无肺栓塞的患者,应考虑PTTM这一诊断。