Pulmonary tumor thrombotic microangiopathy:1 case report and review of the literatures

FENG Mengjie; Qiu Chen; XIAN Zhihong

doi:10.3969/j.issn.1671-6450.2018.03.019

Abstract：Objective To improve the clinical recognition of pulmonary tumor thrombotic microangiopathy(PTTM). Methods One case of clinically diagnosed of PTTM admitted to Shenzhen People's Hospital in July 2017 was reported,and 27 cases of antemortem diagnosis reported in the literatures were analyzed retrospectively. Results Among the 27 PTTM ca-ses reported in the literatures, the main clinical manifestation of 22 cases (81.5%) were progressive dyspnea, 18 cases (66.7%) with pulmonary hypertension,16 cases(59.3%) with dry cough and 17 cases(63.0%) with hypoxemia. 10 ca-ses (37.0%) had an increase in D dimer and 5 cases(18.5%) had an increase in tumor markers,and coagulation disorder was observed in 4 cases (14.8%). On chest CT,13 cases(48.1%) showed multiple nodules,10 cases(37.0%) showed ground glass opacity,and 6 cases (22.2%) showed lobular septa thickening. 10 cases (37.0%) received chemotherapy a-lone or in combined with anticoagulation,anti-pulmonary hypertension or imatinib,7 cases(25.9%) received anti-pulmonary hypertension therapy alone or in combined with glucocorticoid and anticoagulant therapy,3 cases(11.1%) were treated with imatinib alone,1 case (3.7%) was treated with omalizumab alone and 6 cases (22.2%) were treated with routine care. 8 patients (29.6%) died within 2 weeks,10 patients (37.0%) had a survival ranging from 2 weeks to 3 months,7 patients (25.9%) had a survival ranging from 3 months to 12 months. 1 patient (3.7%) survived for more than 2 years,and 1 pa-tient (3.7%) improved after treatment and no further follow-up was obtained. Conclusion PTTM presents clinically as pro-gressive dyspnea and dry cough,accompanied by pulmonary hypertension and coagulation dysfunction. Serum D dimer level and tumor markers may be elevated. Chest CT mainly shows centrilobular nodules, ground glass opacity and lobular septa thickening. CTPA always shows negative results. Confirmed diagnosis should be based on histopathology. There is currently no standard treatment for PTTM and chemotherapy or imatinib treatment may be a choice.