Abstract：
Thrombotic thrombocytopenic purpura (TTP) during pregnancy is life-threatening. We encountered two pregnant women with immune-mediated TTP (iTTP). A 40-year-old primigravida woman was referred at 19 gestational weeks (GWs) owing to iTTP. She received plasma exchange (PE) and steroid therapies and delivered a live infant at 27 GWs by cesarean delivery. A 29-year-old primigravida woman was referred owing to intrauterine fetal death and thrombocytopenia at 20 GWs. She was diagnosed with iTTP and received PE therapy. She required additional PE and steroid therapies owing to relapse. Before her second pregnancy, she received prednisolone and hydroxychloroquine according to the therapy for systemic lupus erythematosus (SLE). She had induced labor at 37 GWs owing to decrease plasma level of a disintegrin-like and metalloproteinase with thrombospondin type 1 motif 13 (ADAMTS13) activity. Close monitoring of plasma ADAMTS13 activity level and treatments for underlying SLE may prevent iTTP relapse and lead to a good prognosis.
摘要：
妊娠期血栓性血小板减少性紫癜（TTP）危及生命。我们遇到了两名患有免疫介导的TTP（iTTP）的孕妇。由于iTTP，一名40岁的primigravida妇女在19孕周（GWs）被转诊。她接受了血浆置换（PE）和类固醇治疗，并在27GWs时通过剖宫产分娩了一名活婴儿。一名29岁的primigravida妇女因20GWs时宫内胎儿死亡和血小板减少症而被转诊。她被诊断为iTTP并接受了PE治疗。由于复发，她需要额外的PE和类固醇治疗。在她第二次怀孕之前，根据系统性红斑狼疮(SLE)的治疗方案,她接受了泼尼松龙和羟氯喹治疗.由于具有血小板反应蛋白1型基序13（ADAMTS13）活性的解整合素样和金属蛋白酶的血浆水平降低，她在37GWs下引产。密切监测血浆ADAMTS13活性水平和潜在SLE的治疗可预防iTTP复发并导致良好预后。
