Liu Fang, Zhu Lanyan, Chen Ping, Shi Zhihui, Liu Shaokun
Department of Respiration, Second Xiangya Hospital, Central South University, Changsha 410011, China.
Zhong Nan Da Xue Xue Bao Yi Xue Ban. 2013 Sep;38(9):971-6. doi: 10.3969/j.issn.1672-7347.2013.09.018.
To explore the clinical manifestations, diagnosis and treatment of pulmonary thromboembolism associated with protein C (PC)/protein S (PS) deficiency. Two male patients 29 and 26 years old diagnosed with PC deficiency and/or PS deficiency 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 flow 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, vitamin 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.
探讨蛋白C(PC)/蛋白S(PS)缺乏症合并肺血栓栓塞症的临床表现、诊断及治疗。回顾性分析2例分别为29岁和26岁诊断为PC缺乏症和/或PS缺乏症的男性患者,并复习相关文献。最常见症状为下肢疼痛伴胸痛或视力下降。彩色多普勒血流成像(CDFI)显示下肢深静脉血栓形成。多层螺旋CT血管造影(CTA)显示肺栓塞。患者1血清同型半胱氨酸(HCY)水平升高,血浆PC/PS含量下降至PC为57.4%,PS为28.9%,患者2的PS为33.4%。观察到常规抗凝反应不佳。诊断PC/PS缺乏症后,加用维生素B6和B12抗凝治疗,患者症状明显改善。对于40岁以前无明显诱因反复发生下肢深静脉血栓形成和肺栓塞的年轻患者,应考虑先天性易栓症。血浆PC/PS浓度或活性对诊断和治疗有很大帮助。