妊娠相关血栓性微血管病患者的临床和病理特征

Clinical and pathological features of patients with pregnancy-related thrombotic microangiopathy

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。

Objective： To investigate the clinical features,morphological characteristics,treatment and prognosis of patients with pregnancy-related thrombotic microangiopathy（ TMA）. Methodology： From Jan,2004 to April,2014,eleven cases were diagnosed as pregnancy-related TMA. Their clinical and pathological features,treatment and prognosis were analyzed retrospectively. Results： They were 2 cases of thrombotic thrombocytopenic purpura（ TTP）,5cases of hemolytic uremic syndrome（ HUS）,one hemolysis,elevated liver enzymes,low platelet count syndrome（ HELLP syndrome）,and 3 cases of pre-eclampsia The onset time was in the late of pregnancy or in one week of postpartum. All of the pregnant women were alive,but 5 cases had stillbirth. The typical clinical manifestations of patients were acute kidney injury（ AKI）,anemia and thrombocytopenia. There were different degrees of endothelial dysfunction in 11 cases. 8 patients received renal biopsy. The histological examination showed interstitial and blood vessel lesions in 7 patients,mainly involving the interlobular arteries,arterioles and afferent arterioles. The major lesions included fibrinoid necrosis,thrombosis,endothelial cell swelling,and arterial ＂onion skin-like ＂ change. All of the patients received continuous renal replacement therapy,5 HUS patients received plasma exchange,and 2 TTP patients received plasma transfusion and immunosuppressive therapy. The renal function of the HUS and HELLP patients recovered completely, and the remaining patients come into the chronic renal insufficiency, two cases receiving maintenance hemodialysis. Conclusion： Pregnancy-related TMA is a harmful disease which threatened the health of pregnant women and fetal life. Despite the molecular mechanisms of pregnancy-related TMA is difference,endothelial damage play a critical role. Distinguishing the cause of the disease,giving timely and effective individualized treatment is one of the important factors which affect the prognosis of pregnancy-related TMA.