为提高对蛋白C(PC)/蛋白S(PS)缺乏症相关的肺动脉血栓栓塞症临床表现、诊治方法的认识，本研究分析了湘雅二医院收治的2例PC/PS缺乏相关肺动脉血栓栓塞患者的临床特点，并进行相关文献复习。两位男性患者年龄分别为29，26岁，临床症状主要表现为自发性反复双下肢肿痛，或胸闷气促、胸痛，或视力下将。血管彩超示下肢静脉血栓形成；肺部血管造影(CTA)示双下肺动脉栓塞；血清同型半胱氨酸浓度升高，血浆PC/PS总量明显降低，病例1为PC 57.4%，PS 28.9%；病例2为PS 33.4%。患者行常规抗凝治疗疗效差，予以维生素B6、维生素B12联合抗凝治疗后症状改善。中青年无明显诱因的反复发作性下肢深静脉血栓形成及肺血栓栓塞，应考虑先天性易栓症可能性，血浆PC，PS浓度及活性检测具有重要的诊断治疗意义。%To explore the clinical manifestations, diagnosis and treatment of pulmonary thromboembolism associated with protein C (PC)/protein S (PS) deifciency. Two male patients 29 and 26 years old diagnosed with PC deifciency and/or PS deifciency were retrospectively analyzed and related literatures were reviewed. The most common symptoms were pain in the lower limbs with chest pain or decreased vision. Color dopper lfow imaging (CDFI) showed lower deep venous phlebothrombosis. Multislice CT angiography (CTA) revealed pulmonary embolism. The level of serum homocysteine (HCY) increased and the level of plasma PC/PS content decreased to PC 57.4%, and PS 28.9%in patient 1, while PS 33.4%in patient 2. Poor routine anticoagulant response was observed. After the diagnosis of PC/PS deficiency, vitiamin B6 and B12 anticoagulant therapy was added, and the symptoms in the patients improved significantly. Congenital thrombophilia should be taken into consideration for young patients with lower deep venous thrombosis and pulmonary embolism which occur recurrently without obvious predisposing causes before 40. Plasma PC/PS concentrations or activity help a lot in the diagnosis and treatment.
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