临床诊断肺肿瘤血栓栓塞性微血管病一例并文献复习  

A case report of pulmonary tumor thrombotic microangiopathy and review of the literature

作　　者：曾庆萃 龙怀聪 王玉霞 ZENG Qingcui;LONG Huaicong;WANG Yuxia(Geriatric ICU, Sichuan Academy of Medical Science ＆ Sichuan Provincial People＇s Hospital, Chengdu, Sichuan 610072, P. R. China)

机构地区：[1]四川省医学科学院.四川省人民医院老年重症监护室,四川成都610072

出　　处：《中国呼吸与危重监护杂志》2018年第3期279-284,共6页Chinese Journal of Respiratory and Critical Care Medicine

基　　金：四川省干保基金(川干研2016-205)

摘　　要：目的探讨肺肿瘤血栓栓塞性微血管病(PTTM)的临床特征、诊断、治疗及预后。方法报道1例临床诊断的PTTM患者。通过万方数据库和Pub Med检索1990~2017年国内外报道的PTTM相关文献,分析PTTM相关的临床资料。结果患者,女,62岁。以干咳、气促及咯血为主要症状。住院期间气促进行性加重,并出现低氧。心脏彩超提示肺动脉高压,右心膨隆,将左心室压缩成D字型。肺部计算机体层摄影血管造影(CTA)排除了肺栓塞。外周血查见大量晚幼红细胞和红细胞碎片及进行性下降的血小板提示患者存在血栓性微血管疾病。CT提示椎骨及胸骨广泛肿瘤转移病灶。根据临床表现及辅助检查临床诊断为PTTM。患者经过积极呼吸支持及抗凝、复苏等相关治疗,仍于住院第5 d死亡。文献复习共检索到PTTM完整病例92例。气促及咳嗽为患者入院的主要原因。在住院过程中患者均存在不同程度的低氧。肺部影像学表现缺乏特异性。所有患者肺部CTA均未见明显异常,心脏彩超或Swan-Ganz气囊漂浮导管提示不同程度的肺动脉高压,部分患者合并弥漫性血管内凝血和(或)微血管病性溶血性贫血。PTTM的确诊有赖于组织学证据,但由于病情进展迅速,许多患者不能耐受肺活检,故国外通常依靠死后尸体解剖获得病理学证据。PTTM的治疗主要包括呼吸支持治疗、抗凝、降肺动脉压等对症治疗,以及对原发或转移肿瘤的化疗及靶向治疗。PTTM的预后极差,几乎所有的患者在出现明显症状后均短期内死亡(48 h到3个月)。结论出现进行性气促伴低氧、肺动脉压力增高,而肺部CTA正常,有肿瘤病史或发现有肿瘤转移病灶的患者应该考虑到PTTM的可能。Objective To investigate the clinical features, diagnosis, treatment and prognosis of pulmonary tumor thrombotic microangiopathy（PTTM）. Methods A patient with PTTM was reported. Literatures about PTTM searched by Wan Fang databases and Pub Med were reviewed for its clinical characteristics. Results A 62-year-old female was admitted with chief complaint of dry cough, dyspnea and hemoptysis. Progressive dyspnea, pulmonary hypertension and hypoxemia occurred during hospitalization. Computed tomography angiography（CTA） of the lung excluded pulmonary embolism. Peripheral blood appearing a large number of late erythroblasts and erythrocyte debris and progressively decreasing platelets suggested that the patient suffer from thrombotic microvascular disease. CT showed widely metastatic lesions at the vertebrae and sternum. On the basis of above clinical characteristics, PTTM was diagnosed clinically. Although the patient accepted respiratory support therapy, anticoagulation therapy and resuscitation, she still died 5 days later after hospitalization. Literatures about PTTM with complete clinical information were reviewed. A total of 92 PTTM cases were reviewed and the main reasons of these patients admitted were progressive dyspnea and chronic cough. During hospitalization, they all suffered varying degrees of hypoxia,while radiological findings of the lungs lack specificity. No abnormal sighs were found by lung CTA. The results of ultrasonic cardiography or the Swan–Ganz catheter indicated varying degrees of pulmonary hypertension, some patients were proved with disseminated intravascular coagulation and/or microangiopathic hemolytic anemia.The definite diagnosis of PTTM depended on the histologic evidence which were often obtained from post-mortem examination, because many patients couldn＇t tolerate the lung biopsy due to rapid aggravation. The treatment of PTTM included respiratory support therapy, anticoagulation therapy, antipulmonary hypertension and the chemotherapy of primary or metastatic tumour. The

关 键 词：肺肿瘤血栓栓塞性微血管病 微血管病性溶血性贫血 肺动脉高压 肿瘤转移 

分 类 号：R734.2[医药卫生—肿瘤]