Success and limitations of plasma treatment in pregnant women with congenital thrombotic thrombocytopenic purpura
Success and limitations of plasma treatment in pregnant women with congenital thrombotic thrombocytopenic purpura
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DOI：
10.1111/jth.15064
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发表时间：
2020-10-15
影响因子：
10.4
通讯作者：
Matsumoto, Masanori
中科院分区：
文献类型：
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作者：
Sakai, Kazuya;Fujimura, Yoshihiro;Matsumoto, Masanori
背景先天性血栓性血小板减少性紫癜（cTTP），又称Upshaw-Schulman综合征，是一种极其罕见的遗传性疾病。妊娠被认为是cTTP患者TTP发作的触发因素。目的探讨妊娠cTTP患者的理想处理方法。患者/方法我们在日本cTTP登记处找到了21名有生育史的患者（38次怀孕）。比较两组胎儿结局：1组（n = 12），通过adamts13基因分析确诊cTTP后妊娠；2组（n = 26），确诊为cTTP前已怀孕。结果1组大多数患者在分娩前密切监测ADAMTS13活性。1组10例妊娠，妊娠期间预防性输注新鲜冷冻血浆（FFP）补充ADAMTS13。2组23例妊娠未给予预防性输注FFP，仅3例妊娠进行了FFP试验输注。组1的活产率显著高于组2 （91.7% vs 50.0%, P = 0.027）。未输注FFP的孕妇在妊娠20周后的胎儿存活率显著下降。妊娠20周时，组1输注FFP剂量普遍高于5 mL/kg/周。结论孕妇应尽早开始FFP输注量大于5 mL/kg/周。然而，即使有这些输液，怀孕前反复发作TTP的患者也可能难以成功分娩。重组ADAMTS13产品可能成为妊娠cTTP患者新的治疗选择。
Background Congenital thrombotic thrombocytopenic purpura (cTTP), otherwise known as Upshaw-Schulman syndrome, is an extremely rare hereditary disease. Pregnancy is identified as a trigger for TTP episodes in patients with cTTP. Objectives To investigate the ideal management of pregnant patients with cTTP. Patients/Methods We identified 21 patients with a reproductive history (38 pregnancies) in a Japanese cTTP registry. Fetal outcomes were compared between two groups: group 1 (n = 12), pregnancy after diagnosis of confirmed cTTP byADAMTS13gene analysis; and group 2 (n = 26), pregnancy before diagnosis of confirmed cTTP. Results In group 1, ADAMTS13 activity was closely monitored until delivery in most cases. Among 10 pregnancies in group 1, prophylactic fresh frozen plasma (FFP) infusions during pregnancy were performed to replenish ADAMTS13. In group 2, prophylactic FFP infusions were not administrated in 23 pregnancies and FFP test infusions were performed in only three pregnancies. The live birth rate of group 1 was significantly higher than that of group 2 (91.7% vs 50.0%, respectively,P = .027). The fetal survival rates of women without FFP infusions were dramatically decreased after 20 weeks of gestation. The FFP infusion dosage in group 1 was generally higher than 5 mL/kg/wk by 20 weeks of gestation. Conclusions Our results indicate that FFP infusions of more than 5 mL/kg/wk should be initiated as soon as patients become pregnant. However, even with these infusions, patients with repeated TTP episodes before pregnancy might have difficulty giving birth successfully. Recombinant ADAMTS13 products might be new treatment options for pregnant patients with cTTP.