目的观察肺肿瘤血栓性微血管病(PTTM)的临床、影像学及病理特点,提高临床医生对该病的认识。方法回顾性分析南京大学医学院附属鼓楼医院 2013 年 10 月至 2016 年 8 月根据临床、影像学及病理特点确诊的 3 例 PTTM 的临床资料,总结其临床、影像学以及病理特征。结果3 例 PTTM 中男 2 例,女 1 例,年龄 29~51 岁,均为亚急性起病,主要临床表现为干咳、进行性呼吸困难。实验室检查示 D-二聚体明显增高,血气分析提示低氧血症。超声心动图肺动脉收缩压中重度增高。高分辨率 CT 均表现为双肺弥漫性小结节影、小叶间隔增厚。正电子发射计算机断层显像均证实原发肿瘤部位。3 例患者原发病均为胃癌,其中 2 例经支气管镜肺活检证实胃癌肺转移,另一例骨活检证实胃癌伴骨转移。患者入院时均未明确诊断为肿瘤,病情进展迅速,于入院后 2 周内死亡。结论PTTM 临床症状无特异性,一旦肿瘤患者出现进行性的呼吸困难及血液高凝状态,出现不明原因的肺动脉高压,而 CT 下肺动脉造影未显示肺栓塞时,应警惕 PTTM 的可能。PTTM 诊断困难,容易漏诊、误诊,缺乏有效的治疗手段,预后极差。
Citation:
张娟娟, 桂贤华, 蔡后荣, 肖永龙, 邱玉英, 孟凡青, 付尧. 三例肺肿瘤血栓性微血管病的临床、影像及病理分析. Chinese Journal of Respiratory and Critical Care Medicine, 2018, 17(5): 509-513. doi: 10.7507/1671-6205.201707049
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- 1. von Herbay A, Illes A, Waldherr R, et al. Pulmonary tumor thrombotic microangiopathy with pulmonary hypertension. Cancer, 1990, 66(3): 587-592.
- 2. Uruga H, Fujii T, Kurosaki A. Pulmonary tumor thrombotic microangiopathy: a clinical analysis of 30 autopsy cases. Intern Med, 2013, 52(12): 1317-1323.
- 3. Chinen K, Tokuda Y, Fujiwara M. Pulmonary tumor thrombotic microangiopathy in patients with gastric carcinoma: an analysis of 6 autopsy cases and review of the literature. Pathol Res Pract, 2010, 206(10): 682-689.
- 4. Higo K, Kubota K, Takeda A. Successful antemortem diagnosis and treatment of pulmonary tumor thrombotic microangiopathy. Intern Med, 2014, 53(22): 2595-2599.
- 5. 桂贤华, 蔡后荣. 肺肿瘤血栓性微血管病. 中华结核和呼吸杂志, 2016, 39(7): 546-547.
- 6. Higashi A, Dohi Y, Uraoka N. The potential role of inflammation associated with interaction between osteopontin and CD44 in a case of pulmonary tumor thrombotic microangiopathy caused by breast cancer. Intern Med, 2015, 54(22): 2877-2880.
- 7. Takahashi F, Kumasaka T, Nagaoka T. Osteopontin expression in pulmonary tumor thrombotic microangiopathy caused by gastric carcinoma. Pathol Int, 2009, 59(10): 752-756.
- 8. 陈久玲, 刘越, 尹新华. 肺肿瘤血栓性微血管病研究新进展. 医学研究杂志, 2016, 45(8): 8-10.
- 9. 李羲, 张劭夫, 钱桂生. 关注肺肿瘤血栓性微血管病. 中华肺部疾病杂志(电子版), 2013, 6(6): 1-3.
- 10. Fukada I, Araki K, Kobayashi K, et al. Imatinib could be a new strategy for pulmonary hypertension caused by pulmonary tumor thrombotic microangiopathy in metastatic breast cancer. Springerplus, 2016, 5(1): 1582.