肺肿瘤血栓性微血管病2例报道并文献复习  

Pulmonary tumor thrombotic microangiopathy:2 cases report and literature review

作　　者：占丰富[1] 张孝斌[1] 黄茂宏[1] 王淼[1] 杜艳萍[1] 曾惠清[1] Zhan Fengfu;Zhang Xiaobin;Huang Maohong;Wang Miao;Du Yanping;Zeng Huiqing(Department of Pulmonary and Critical Care Medicine,Zhongshan Hospital,Xiamen University,Fujian Province,Xiamen 361004,China)

机构地区：[1]厦门大学附属中山医院呼吸与危重症医学科,福建厦门361004

出　　处：《疑难病杂志》2022年第7期736-741,共6页Chinese Journal of Difficult and Complicated Cases

基　　金：国家自然科学基金面上项目(82170103);福建省卫生健康科技计划中青年骨干人才培养项目(2020GGB057);厦门市科技局卫生指导性项目(3502Z20214ZD1043)。

摘　　要：目的探讨肺肿瘤血栓性微血管病(PTTM)的临床特征、诊断、治疗及预后,以提高对PTTM的认识。方法报道2020年9月和10月厦门大学附属中山医院收治的2例PTTM病例,并对截止2022年1月国内外文献报道的来自中国的PTTM病例进行回顾性分析。结果共检索发现中国的PTTM病例报道文献14篇20例,结合笔者报道的2例,共22例。其中,最常见的原发肿瘤是胃癌(59.1%),最多见临床特征为渐进性加重的气促(90.9%)。全部病例D-二聚体水平异常,肺动脉CTA未见明显异常,心脏彩色超声提示中位肺动脉压为64.0(57.5,70.3)mmHg。PTTM患者病情进展迅速,大部分不能耐受肺活检,生前临床诊断19例,经支气管镜下肺活检确诊2例,死后尸检确诊1例。PTTM的关键治疗为针对原发肿瘤的治疗,预后极差,患者诊断后的中位生存期为7.5(3.0,12.3)d。结论PTTM是一种罕见的病理诊断困难、预后极差的临床病理综合征。当癌症患者出现渐进性加重的气促,以及高凝状态、肺动脉高压,但肺动脉CTA未发现肺栓塞时,应该警惕PTTM。生前早期识别和诊断并针对原发肿瘤的治疗是改善预后的关键。Objective To explore the clinical features,diagnosis,treatment and prognosis of pulmonary tumor thrombotic microangiopathy(PTTM)in order to improve the understanding of PTTM.Methods Two PTTM cases admitted to Zhongshan Hospital Affiliated to Xiamen University in September and October 2020 were reported,and a retrospective analysis of PTTM cases from China reported in domestic and foreign literature as of January 2022 was conducted.Results A total of 14 PTTM case reports in China with 20 cases were found in the search.Combined with the 2 cases reported by the author,a total of 22 cases were found.Among them,the most common primary tumor was gastric cancer(59.1%),and the most common clinical feature was progressive worsening shortness of breath(90.9%).D-dimer levels were abnormal in all cases,pulmonary artery CTA showed no obvious abnormality,and the median pulmonary artery pressure was 64.0(57.5,70.3)mmHg indicated by cardiac color ultrasound.The condition of PTTM patients progressed rapidly,and most of them could not tolerate lung biopsy.There were 19 cases diagnosed by clinical diagnosis before death,2 cases diagnosed by lung biopsy through bronchoscopy,and 1 case diagnosed by postmortem autopsy.The key treatment of PTTM is the treatment of the primary tumor,and the prognosis is extremely poor.The median survival time after diagnosis is 7.5(3.0,12.3)days.Conclusion PTTM is a rare clinicopathological syndrome with extremely poor prognosis and difficult pathological diagnosis.PTTM should be vigilant in cancer patients with progressively worsening shortness of breath,hypercoagulability,and pulmonary hypertension,but no pulmonary embolism on pulmonary artery CTA.Early identification and diagnosis before life and treatment targeting the primary tumor are the keys to improving prognosis.

关 键 词：肺肿瘤血栓性微血管病 肺动脉高压 肿瘤 诊断 治疗 

分 类 号：R734.2[医药卫生—肿瘤]