4例妊娠合并血栓性微血管病的临床观察

Thrombotic Microangiopathies Complicating Pregnancy:A Report of Four Cases

目的:通过4例妊娠合并血栓性微血管病[TMA,其中溶血性尿毒症综合征(HUS)和血栓性血小板减少性紫癜(TTP)各2例]患者的临床资料分析,探讨其诊断与治疗。方法:通过病史记录,对临床、实验室检查和(或)病理诊断的4例TMA资料进行回顾性分析。结果:4例患者中3例初产妇,1例经产妇。3例患者孕龄32~37周,均死胎,母亲均存活;1例患者24周并发自然流产,入院后死亡。剖宫产1例,阴道分娩2例。实验室检查:4例患者Hb29.0~57.0g/L,Plt14×109/L^40×109/L,外周血片中均见破碎红细胞;4例患者均有肾功能损害,2例患者分娩后即无尿,其中1例患者行肾脏穿刺,病理报告符合HUS。存活的3例患者都接受血浆、洗涤红细胞、血小板治疗和(或)持续肾替代治疗(CRRT)。2例HUS患者行血液透析治疗,1例患者行TTP血浆置换。结论:妊娠合并TTP、HUS的母儿死亡率高,内皮细胞损伤可能是继发导致微血管血栓形成的一个重要原因,产科医生必须对此病提高警惕,早期诊断、早期输血浆或血浆置换,能提高母儿生存率。

Objective：To explore the diagnosis and treatment of microangiopathies （TMA） complicating pregnancies through case review.Methods：Four cases of TMA complicating pregnancy were retrospectively analysed,including 2 hemolytic uremic syndrome（HUS）and 2 thrombotic thrombocytopenic purpura （TTP）. Medical records,clinical pictures,laboratory and pathologic data were reviewed.Results：Among the 4 cases,3 were nulliparous,one multipara. 1 had spontaneous abortion and died 8 hours after admission into hospital. The other 3 had fetal death around 32w to 37w,one of whom delivered by Cesarean section,the other 2 delivered vaginally. All 4 cases were anemia （Hb ranged from 29.0 to 57.0g/L） and all had abnormal fragmented red cells. The platelet count ranged from 14 to 40 ×10^9/L. All had oliguria with renal dysfunction. 2 had acute anuria after delivery. Renal biopsy in 1 case showed HUS. Treatment included fresh-frozen plasma,MAP,platelets and /or continued renal replacement therapy（CRRT）.Conclusions：TTP and HUS complicated pregnancy is associated with high maternal mortality and morbidity. The endothelial dysfunction appears to be an important reason. Early diagnosis and plasmapheresis can improve the prognosis of mother and fetus.