妊娠合并血栓性血小板减少性紫癜2例并文献复习  

Pregnancy complicated with thrombotic thrombocytopenic purpura:two cases report and literature review

作　　者：谢江燕 王亚菲 贺芳[1,2] Jiangyan Xie;Yafei Wang;Fang He(Department of Obstetrics and Gynecology,Guangdong Provincial Key Laboratory of Obstetrics Diseases,Guangdong-Hong Kong-Macao Greater Bay Area Higher Education Joint Laboratory of Maternal-Fetal Medicine,Guangdong Engineering and Technology Research Center for Maternal-Fetal Medicine,The Third Affiliated Hospital,Guangzhou Medical University,Guangzhou 510150,Guangdong Province,China;Guangdong Provincial Clinical Research Center of Obstetrics and Gynecology,The Third Affiliated Hospital,Guangzhou Medical University,Guangzhou 510120,Guangdong Province,China;Department of Obstetrics,Hainan Women and Children Medical Center,Haikou 570206,Hainan Province,China;Department of Obstetrics,Guiyang Second People's Hospital,Guiyang 550005,Guizhou Province,China)

机构地区：[1]广州医科大学附属第三医院妇产科,广东省产科重大疾病重点实验室,粤港澳母胎医学高校联合实验室,广东省母胎医学工程技术研究中心,广州510150 [2]广东省妇产疾病临床医学研究中心,广州510120 [3]海南省妇女儿童医学中心产科,海口570206 [4]贵阳市第二人民医院产科,贵阳550005

出　　处：《中华妇幼临床医学杂志（电子版）》2024年第5期556-563,共8页Chinese Journal of Obstetrics & Gynecology and Pediatrics(Electronic Edition)

基　　金：广东省基础与应用基础研究基金区域联合基金重点项目(2021B1515120070)。

摘　　要：目的探讨妊娠合并血栓性血小板减少性紫癜(TTP)患者的诊治。方法选择2022年9月3日和2024年2月17日广州医科大学附属第三医院收治的2例妊娠合并TTP患者(患者1、2)为研究对象。回顾性分析其发病特点、实验室检查结果、诊治过程及预后。检索国内外数据库中,关于妊娠合并TTP研究的相关文献,总结妊娠合并TTP患者的临床表现、诊断与治疗措施。本研究遵循的程序符合2013年修订的《世界医学协会赫尔辛基宣言》要求。结果①患者1因“停经36+3周,双下肢水肿2周,发现血压升高2 d”入院,既往合并系统性红斑狼疮(SLE),于孕龄为36+3孕周时出现血小板计数(PLT)严重降低、溶血性贫血,考虑“狼疮危象”,采取急诊剖宫产术分娩。根据ADAMTS13活性为0.1%的检测结果,患者1被确诊为SLE合并TTP。对患者1采取输注血小板、红细胞,以及血浆置换、类固醇激素治疗后,救治成功。②患者2因“剖宫产术分娩后7+h,肝酶升高及PLT降低6+h”入院。其于孕龄为36^(+4)孕周时,因重度子痫前期(sPE)进行急诊剖宫产术分娩,术后PLT降低、肝酶升高,疑诊为溶血、肝酶升高、血小板减少(HELLP)综合征,PLT进一步降低,伴溶血性贫血,根据ADAMTS13活性及抑制物检测结果,患者2被确诊为TTP。对其采取血浆置换、输血及血液净化治疗后,病情缓解出院,1个月后随访肾功能恢复正常。③按照本研究设定的文献检索策略,检索到97篇报道妊娠合并TTP研究文献,纳入125例妊娠合并TTP患者,加上本研究2例(患者1、2),共计127例妊娠合并TTP患者中,63例(49.6%)表现为TTP三联征、8例(6.3%)为五联征;58例(45.7%)进行ADAMTS13活性检测患者中,48例(82.8%)为活性重度降低;100例(78.7%)采取血浆置换治疗患者中,25例(25.0%)死亡,未采取血浆置换治疗的27例(21.3%)患者中,12例(44.4%)死亡。结论妊娠是导致孕产妇发生TTP的诱因之一,对临床合并微血管病性�Objective To explore the diagnosis and treatment of pregnancy complicated with thrombotic thrombocytopenic purpura(TTP).Methods Two cases of pregnancy complicated with TTP admitted to the Third Affiliated Hospital of Guangzhou Medical University on September 3,2022,and February 17,2024(patient 1 and patient 2)were selected as the subjects of this study.A retrospective analysis of their clinical characteristics,laboratory test results,diagnosis and treatment processes,and outcomes was conducted.Additionally,literature related to TTP in pregnancy from domestic and international databases was searched to summarize the clinical manifestations,diagnosis,and treatment of pregnancy complicated with TTP.The procedures followed in this study complied with the requirements of the 2013 revision of the World Medical Association Declaration of Helsinki.Results①patient 1 was admitted due to"amenorrhea at 36+3 weeks,both lower limb edema for 2 weeks,and elevated blood pressure for 2 days",with a history of systemic lupus erythematosus(SLE).At 36+3 weeks of gestation,she developed severe decrease of platelet count(PLT)and hemolytic anemia,which led to the consideration of"lupus crisis",then an emergency cesarean section was performed.According to the activity of ADAMTS13 was measured to be 0.1%,patient 1 was confirmed the diagnosis of SLE complicated with TTP.After platelet transfusion,red blood cell transfusion,plasma exchange,and corticosteroid therapy for patient 1,she was successfully treated.②Patient 2 was admitted due to"elevated liver enzymes and thrombocytopenia for 6+h after cesarean section".At 36^(+4) weeks of gestation,she underwent an emergency cesarean section due to severe preeclampsia(sPE).Postoperatively,she experienced a decrease in PLT and an increase in liver enzymes,suspected to have hemolysis,elevated liver enzymes,low platelet count(HELLP)syndrome.Subsequently,the PLT further decreased,accompanied by h emolytic anemia.According to the activity and inhibitor of ADAMTS13 test results,a diagnosis of TTP w

关 键 词：紫癜 血栓性血小板减少性 妊娠 血浆置换 ADAMTS13蛋白质 贫血 溶血性 孕妇 

分 类 号：R714.2[医药卫生—妇产科学]