Hsu H W, Belfort M A, Vernino S, Moake J L, Moise K J
Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, Texas, USA.
Obstet Gynecol. 1995 May;85(5 Pt 2):839-43. doi: 10.1016/0029-7844(94)00407-5.
Thrombotic thrombocytopenic purpura is an infrequent but devastating complication of pregnancy, often difficult to differentiate from severe preeclampsia and the syndrome of hemolysis, elevated liver enzymes, and low platelets (HELLP). To our knowledge, the combination of thrombotic thrombocytopenic purpura and hepatic vein thrombosis has not been reported previously in pregnancy.
A 33-year-old woman, a multigravida, was delivered at 36 weeks' gestation because of pregnancy-induced hypertension and HELLP syndrome. Postpartum, the patient became obtunded, disoriented, and anuric. Her laboratory values revealed a Coombs-negative, microangiopathic hemolytic anemia, modestly abnormal coagulation studies, and thrombocytopenia. She also had elevated liver enzymes, lactate dehydrogenase, and creatinine. The largest plasma von Willebrand factor multimeric forms were relatively decreased in her ethylenediaminetetra-acetic acid-platelet-poor plasma. A diagnosis of thrombotic thrombocytopenic purpura was made. The patient received plasma exchange, hemodialysis, blood transfusion, and glucocorticoids. She responded to therapy, but was later noted to have increasing hepatosplenomegaly, rising levels of bilirubin, and elevated alkaline phosphatase. A Doppler study and magnetic resonance imaging demonstrated the absence of flow in the middle and left hepatic veins, secondary to thrombosis (Budd-Chiari syndrome). She was maintained on warfarin therapy and was discharged on postpartum day 50.
The early recognition of thrombotic thrombocytopenic purpura is crucial for the initiation of appropriate treatment as soon as possible. The avoidance of platelet transfusion and early plasma-exchange therapy may be lifesaving.
血栓性血小板减少性紫癜是妊娠罕见但严重的并发症，常难以与重度子痫前期及溶血、肝酶升高和血小板减少综合征（HELLP）相鉴别。据我们所知，妊娠合并血栓性血小板减少性紫癜和肝静脉血栓形成此前尚未见报道。
一名33岁经产妇，因妊娠高血压和HELLP综合征于孕36周分娩。产后，患者出现意识不清、定向障碍及无尿。实验室检查结果显示为抗人球蛋白试验阴性的微血管病性溶血性贫血、凝血检查轻度异常及血小板减少。她还存在肝酶、乳酸脱氢酶及肌酐升高。其乙二胺四乙酸抗凝血浆中最大的血管性血友病因子多聚体形式相对减少。诊断为血栓性血小板减少性紫癜。患者接受了血浆置换、血液透析、输血及糖皮质激素治疗。她对治疗有反应，但后来发现肝脾肿大加重、胆红素水平升高及碱性磷酸酶升高。多普勒检查和磁共振成像显示肝中静脉和肝左静脉因血栓形成而无血流信号（布加综合征）。她接受华法林治疗，产后第50天出院。
尽早识别血栓性血小板减少性紫癜对于尽快开始适当治疗至关重要。避免输注血小板及早期进行血浆置换治疗可能挽救生命。
