肺肿瘤血栓性微血管病3例并文献复习

Pulmonary tumor thrombotic microangiopathy:report of 3 cases and literature review

目的提高临床医生对肺肿瘤血栓性微血管病(PTTM)的早期识别能力和诊断水平。方法本文报道了临床诊断的3例PTTM,分析了其临床表现、影像学特征、实验室检查、诊治经过及预后情况。同时对2018年1月至2024年7月英文文献(68篇,84例)和2024年7月以前国内文献(16篇,23例)报道的PTTM病例进行回顾性分析。结果共分析了包含本文3例在内的110例国内外PTTM病例。PTTM的主要临床症状为呼吸困难(90.0%,99/110),最常见的合并肿瘤是胃癌(32.7%,34/104);多有D-二聚体升高(97.3%,71/73)、肺动脉压升高(89.0%,81/91);生前诊断率低(51.8%,57/110),病死率高达87.7%(93/106);出现呼吸困难后的中位生存时间为30 d,确诊PTTM后的中位生存时间为6 d。结论PTTM是一种罕见的恶性肿瘤并发症,临床表现缺乏特异性,生前确诊率低,预后差,病死率高,给临床诊治带来重大挑战。尽早诊断及有效的抗肿瘤治疗是改善PTTM患者预后的关键。

ObjectiveTo improve the early recognition,diagnostic and treatment levels of pulmonary tumor thrombotic microangiopathy(PTTM).MethodsThree cases of PTTM were reported,and their clinical manifestations,imaging findings,laboratory testing,diagnostic and treatment processes,and prognosis were analyzed.A total of 68 foreign literatures reporting 84 cases of PTTM from January 2018 to July 2024,and 16 domestic literatures reporting 23 cases of PTTM prior to July 2024 were retrospectively reviewed.ResultsA total of 110 cases of PTTM,including 3 in the present study were reviewed.Dyspnea(90.0%,99/110)was the dominant manifestation of PTTM,and gastric cancer(32.7%,34/104)was the most common comorbid tumors.Most of PTTM patients had elevated D-dimer(97.3%,71/73)and pulmonary arterial pressure(89.0%,81/91).The diagnostic rate of PTTM before death was relatively low(51.8%,57/110),and the mortality was up to 87.7%(93/106).The median survival of PTTM after the development of dyspnea was 30 d,and that after confirming PTTM was 6 d.ConclusionsPTTM is a rare complication of malignancies showing atypical clinical manifestations,low diagnostic rate before death,poor prognosis,and high mortality.The diagnosis and treatment of PTTM are challenging.Early diagnosis and effective anti-tumor therapy contribute to improve the prognosis of PTTM.