Kobayashi Kana, Murakami Akari, Komatsu Saaya, Nishiyama Kanako, Yamashita Michiko, Sugimori Wakana, Kamei Yoshiaki, Asai Hiroaki, Ogimoto Akiyoshi, Honda Kazuo, Takada Yasutsugu
Dept. of Hepato-Biliary-Pancreatic Surgery and Breast Surgery, Ehime University Graduate School of Medicine.
Gan To Kagaku Ryoho. 2017 Mar;44(3):243-246.
Pulmonary tumor thrombotic microangiopathy(PTTM)caused by pulmonary artery microscopic tumor emboli and fibrocellular and/or fibromuscular proliferation leads to progressive pulmonary hypertension and respiratory failure.The prognosis is extremely poor and most patients die shortly after onset.We report a patient with Stage IV breast cancer and long-term survival who developed PTTM during chemotherapy treatment.A 63-year-old woman with multiple metastases in her cerebellum, bone, lung, and lymph node after left breast conserving surgery started to experience dyspnea and malaise 7 years after the surgery.Two months later, she was urgently admitted to hospital because of respiratory failure and was diagnosed with pulmonary hypertension.However, pulmonary thrombosis and tumor thrombus were not observed.We clinically diagnosed her with PTTM and administered chemotherapy in addition to treatment for pulmonary hypertension.Her medical condition improved gradually and she survived for the subsequent 2 years.When observing progressive hypoxia and pulmonary hypertension without obvious pulmonary embolism findings on imaging, PTTM should be considered.Early diagnosis and immediate induction of chemotherapy for primary disease can improve the survival of patients with PTTM.
肺动脉微小肿瘤栓子以及纤维细胞和/或纤维肌肉增生所导致的肺肿瘤血栓性微血管病(PTTM),会引发进行性肺动脉高压和呼吸衰竭。其预后极差,大多数患者在发病后不久便会死亡。我们报告了一名IV期乳腺癌患者,该患者在化疗期间发生了PTTM,但仍长期存活。一名63岁女性在接受左乳保乳手术后,小脑、骨骼、肺部和淋巴结出现多处转移,术后7年开始出现呼吸困难和不适。两个月后,她因呼吸衰竭紧急入院,被诊断为肺动脉高压。然而,未观察到肺血栓形成和肿瘤血栓。我们临床诊断她患有PTTM,并在治疗肺动脉高压的同时给予化疗。她的病情逐渐好转,并在随后的2年里存活了下来。当在影像学上未发现明显肺栓塞表现却观察到进行性低氧血症和肺动脉高压时,应考虑PTTM。早期诊断并立即对原发疾病进行化疗诱导,可提高PTTM患者的生存率。