Yao Lingling, Qin Jun, Wang Jianing, Liu Jinghua
Center for Coronary Artery Disease (CCAD), Beijing Anzhen Hospital, Capital Medical University, and Beijing Institute of Heart, Lung and Blood Vessel Diseases, Beijing, China.
Cardiac Care Unit (CCU), Renmin Hospital, Hubei University of Medicine, Shiyan, Hubei Province, China.
Medicine (Baltimore). 2025 Jul 25;104(30):e43284. doi: 10.1097/MD.0000000000043284.
Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare malignancy-associated condition characterized by progressively worsening dyspnea, dry cough, hypoxemia, pulmonary hypertension, right-sided heart failure, and sudden death.
A 60-year-old female presented with intermittent dry cough, dyspnea, and chest pain.
The patient was suspected of having PTTM with a pancreatic primary malignancy, based on admission findings including percutaneous oxygen saturation of 88%, respiratory alkalosis on blood-gas analysis, and elevated levels of d-dimer, NT-proBNP, and multiple tumor markers, combined with computed tomography angiography results showing enlarged lymph nodes (mediastinum, bilateral hila, right cardiophrenic angle, retroperitoneum) and a blurred peripancreatic space.
Diagnostic investigations included physical examination, blood-gas analysis, laboratory tests (d-dimer, NT-proBNP, tumor markers), and computed tomography angiography of thoracic/abdominal vessels. (No therapeutic interventions detailed).
The patient experienced 4 episodes of sudden clinical deterioration, suffered cardiac arrest, and died 9 days after admission. Autopsy was declined by the family.
PTTM must be considered in the differential diagnosis for patients presenting with dyspnea and new-onset severe pulmonary hypertension without other obvious etiology, particularly when malignancy is suspected.
肺肿瘤血栓性微血管病（PTTM）是一种罕见的与恶性肿瘤相关的疾病，其特征为进行性加重的呼吸困难、干咳、低氧血症、肺动脉高压、右心衰竭和猝死。
一名60岁女性出现间歇性干咳、呼吸困难和胸痛。
根据入院检查结果，包括经皮血氧饱和度为88%、血气分析显示呼吸性碱中毒、D-二聚体、N末端脑钠肽前体（NT-proBNP）和多种肿瘤标志物水平升高，结合计算机断层血管造影结果显示淋巴结肿大（纵隔、双侧肺门、右心膈角、腹膜后）以及胰腺周围间隙模糊，该患者被怀疑患有PTTM并伴有胰腺原发性恶性肿瘤。
诊断性检查包括体格检查、血气分析、实验室检查（D-二聚体、NT-proBNP、肿瘤标志物）以及胸腹部血管计算机断层血管造影。（未详细说明治疗干预措施）
患者经历了4次突然的临床恶化，发生心脏骤停，并在入院9天后死亡。家属拒绝进行尸检。
对于出现呼吸困难和新发严重肺动脉高压且无其他明显病因的患者，尤其是怀疑患有恶性肿瘤时，鉴别诊断中必须考虑PTTM。
