【摘要】 目的探讨妊娠合并血栓性血小板减少性紫癜(TTP)的早期诊断和治疗。方法回顾分析2006年6月至2009年9月苏州大学附属第一医院5例妊娠合并TTP患者的临床资料。结果 5例均为初产妇,孕中期发病2例,孕晚期3例,入院均诊断HELLP综合征。意识障碍3例,发热3例。5例血红蛋白36.0～73.0g/L,血小板(6～42)×109/L,4例外周血片见破碎红细胞,3例血管性血友病因子裂解蛋白酶(vWF-cp,又名ADAMTS13)活性<5%,其中1例基因突变于9号外显子;1例自身抗体阴性;1例自身抗体阳性,余2例ADAMTS13活性正常。孕晚期2例急诊剖宫产,1例阴道产钳助产;2例新生儿存活,1例死胎。孕中期2例剖宫取胎。1例产妇死亡。5例中2例行血浆置换。结论 TTP无特征性早期临床表现,必须警惕在初产妇尤其是并发HELLP综合征患者出现血栓性微血管病时,不论ADAMTS13活性正常与否,应迅速终止妊娠,紧急血浆置换。更多还原

【Abstract】 Objective To investigate the early diagnosis and management of thrombotic thrombocytopenic purpura (TTP)complicating pregnancy.Methods During the June 2006 to Sep.2009,the medical records of 5 cases with TTP complicating pregnancy were reviewed retrospectively. Results Five cases were all primipara.Two cases occurred in second trimester,the other 3 cases in the third trimester,while diagnosis were made as HELLP(hemolytic elevated liver enzymes,low platelets )syndrome at admission.The clinical feature was all anemia,fever associated with coma in 3 cases.The laboratory findings showed hemoglobin ranged from(36.0～73.0g/L),platelets count ranged from(6～42)×109/L.Four cases had abnormal fragmented red cells on peripheral blood smear. Three cases had ADAMTS13 activity<5% of normal,in which one with gene mutation that occurred in the 9 exon,the another one inhibitor detected. The ADAMTS13 activity were normal value in 2 case.Cesearen section was performed in 2 cases,one case received forceps per vagina,among them 2 neonatal were alive,one stillbirth. Two cases received,plasma exchange One patient died without plasma exchange. Conclusion With increasing awareness,extra-attention must be pay to primipara,or especially patients with HELLP syndrome associated with thrombotic microangiopathy(TMA)No matter ADAMTS13 is positive or negative,while prompt progressive management include early termination of pregnancy and plasma exchange.更多还原