妊娠相关血栓性微血管病患者的临床和病理特征

发布时间：2018-05-02 15:49

本文选题：血栓性微血管病 + 内皮功能　；参考：《肾脏病与透析肾移植杂志》2015年04期

【摘要】：目的:探讨妊娠相关血栓性微血管病(TMA)的临床病理特点、治疗和预后。方法:回顾性分析2004年1月至2014年3月南京军区南京总医院肾脏科11例妊娠相关性TMA患者的临床和病理资料。结果:11例患者中有2例血栓性血小板减少性紫癜(TTP),5例产后溶血尿毒综合症(HUS),1例溶血、肝酶升高、血小板减少(HELLP)综合症,3例重度子痫前期,发病时间为妊娠中晚期或产后1周内,母亲均存活,死胎5例。11例患者均达到急性肾损伤(AKI)3期;内皮功能检测提示,患者存在不同程度的内皮功能损伤。8例患者行肾脏活检病理检查,7例见间质血管病变,主要累及小叶间动脉、小动脉及入球动脉,表现为纤维素样坏死、血栓形成、内皮细胞肿胀、动脉"葱皮样"改变。11例患者均接受连续性血液净化治疗,5例产后HUS患者接受血浆置换,2例TTP患者接受血浆输注及免疫抑制剂治疗,5例产后HUS患者和1例HELLP综合征患者肾功能均恢复正常,其余患者进入慢性肾功能不全,其中2例需维持性血液透析治疗。结论:妊娠相关性TMA是影响产妇肾脏功能和胎儿生命的严重疾病,内皮损伤在妊娠相关性TMA中发挥重要作用;肾脏预后与血管病变程度相关。区分妊娠相关性TMA的病因,进行及时有效的个体化治疗是影响妊娠相关性TMA预后的重要因素。
[Abstract]:Objective: to investigate the clinicopathological features, treatment and prognosis of pregnancy-associated thrombotic microangiopathy (TMA). Methods: the clinical and pathological data of 11 patients with pregnancy-associated TMA in Renal Department of Nanjing General Hospital of Nanjing military region from January 2004 to March 2014 were retrospectively analyzed. Results among 11 patients, 2 patients with thrombotic thrombocytopenic purpura (TTP), 5 patients with postpartum hemolytic uremic syndrome (HUSU), 1 patient with hemolytic uremic syndrome, 1 patient with elevated liver enzyme, 3 patients with severe preeclampsia, the onset time was within 1 week after pregnancy. All the mothers survived, the dead fetus in 5 cases and 11 cases all reached the stage 3 of acute renal injury, and the endothelial function test showed that there were 8 cases with different degrees of endothelial function injury and 7 cases with interstitial vascular disease were detected by renal biopsy and pathological examination. Mainly involving the interlobular artery, arteriole and the artery into the bulb, it is characterized by fibrinoid necrosis, thrombosis and swelling of endothelial cells. All 11 patients received continuous blood purification therapy. 5 patients with postpartum HUS received plasma exchange. 2 patients with TTP received plasma infusion and immunosuppressant therapy. 5 patients with postpartum HUS and 1 patient with HELLP The renal function of the patients returned to normal. The remaining patients entered chronic renal insufficiency, 2 of which required maintenance hemodialysis. Conclusion: pregnancy-associated TMA is a serious disease affecting the renal function and fetal life of pregnant women. Endothelial injury plays an important role in pregnancy-associated TMA, and renal prognosis is related to the degree of vascular lesions. Distinguishing the etiology of pregnancy-associated TMA and timely and effective individualized treatment are important factors affecting the prognosis of pregnancy-associated TMA.
【作者单位】：南京军区南京总医院肾脏科国家肾脏疾病临床医学研究中心全军肾脏病研究所;
【基金】：国家科技支撑计划课题(2013BAI09B04,2015BAI12B05) 江苏省临床医学科技专项(BL2012007)
【分类号】：R714.25

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