关键词: 血栓性血小板减少性紫癜, 血管性血友病因子裂解酶, 血浆置换, 血浆, 围生期, 妊娠结局

Abstract: Objective　To investigate the perinatal management of thrombotic thrombocytopenic purpura (TTP) during pregnancy. Methods　One pregnant woman complicated with TTP was successfully treated in Suqian First People's Hospital, Nanjing Medical University, and 16 cases of pregnancy complicated with TTP were reported. The clinical characteristics, laboratory examination, treatment points, and maternal and infant outcomes were retrospectively analyzed. Results　The mean age of the 16 pregnant patients was 26.18 (20-36), and the gestational age was (29±2) weeks (17⁺⁵～41⁺¹ weeks). Fifteen gestation patients with TTP had typical pentad or triad of thrombocytopenia, fever, nervous system symptoms, renal impairment, and microangiopathic hemolytic anemia. The treatment was mainly plasma exchange or infusion. There were four maternal deaths, and one of them was diagnosed postmortem. Case 15 and 16 were two pregnancies of the same woman; case 15 was diagnosed as the previous pregnancy; case 16 was the second pregnancy; the conditions of perinatal preventive plasma infusion were stable; the pregnancy was terminated by cesarean section at 37⁺² weeks of gestation, with good maternal and infant outcomes. There were nine cases of late pregnancy, including eight cases taking cesarean section, one case (stillbirth) taking forceps assisted delivery, and no natural birth; seven cases had alive neonates. There were seven cases of second trimester, including four cases taking hysterectomy, one case of spontaneous abortion, two case taking rivanol induced labor; no neonates survived. Conclusions　Pregnancy complicated with TTP is rare; early diagnosis and plasma exchange or plasma infusion can effectively improve the patients' prognosis. For pregnant patients with inherited TTP, fresh plasma infusion is also an effective method to prevent the onset.

Key words: Thrombotic thrombocytopenic purpura, Von willebrand factor lyase, Plasmapheresis, Plasma, Perinatal period, Pregnancy outcomes