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Maternal and fetal outcomes of pregnancy occurring after a diagnosis of immune-mediated thrombotic thrombocytopenic purpura

Abstract

Pregnancy is a well-established trigger for a first episode or relapse of immune thrombotic thrombocytopenic purpura (iTTP). Other outcomes of subsequent pregnancy after a diagnosis of iTTP are less well described. We conducted this retrospective cohort study to evaluate maternal and fetal outcomes of pregnancy in women with prior iTTP from the Johns Hopkins Thrombotic Microangiopathy Cohort. Of 168 women in the cohort, 102 were of reproductive age at diagnosis. Fourteen pregnancies (in 9 women) that occurred after the initial iTTP episode were included in the analysis. iTTP relapse occurred in 9 (64%) pregnancies. Out of the 9 instances of relapse, 5 relapses occurred in 2 women. Seven pregnancies (50%) ended in fetal death or miscarriage in the setting of iTTP relapse and three were electively terminated due to fear of relapse. Four pregnancies (50% of the 8 that progressed beyond 20 weeks) were complicated by preeclampsia or HELLP syndrome, which is over ten-fold higher than that of the general population. No maternal deaths occurred. Only 4 pregnancies resulted in live births, of which, 2 were pre-term. Pregnancy in women with prior iTTP is associated with a substantial risk of iTTP relapse and fetal loss. Preeclampsia and HELLP syndrome is also more common than that in the general population. ADAMTS13 monitoring and preemptive therapy may improve pregnancy outcomes, which needs to be evaluated prospectively.

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Funding

This work was supported by a grant from the National Heart Lung and Blood Institute (K99HL150594) and an American Society of Hematology Scholar Award to S. Chaturvedi.

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Correspondence to Shruti Chaturvedi.

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This study was approved by the Johns Hopkins Institutional Review Board as a retrospective study with waiver of informed consent.

Competing interests

S. Chaturvedi reports participation in advisory boards for Alexion, Sanofi Genzyme, Sobi, and UCB. MA Mazepa reports payment for lecture and travel by Sanofi Genzyme. The other authors have no competing interests. S. Chaturvedi and M.A. Mazepa serve on the Board of Directors for the United States Thrombotic Microangiopathy Consortium.

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Brown, J., Potugari, B., Mazepa, M.A. et al. Maternal and fetal outcomes of pregnancy occurring after a diagnosis of immune-mediated thrombotic thrombocytopenic purpura. Ann Hematol 101, 2159–2167 (2022). https://doi.org/10.1007/s00277-022-04936-2

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  • DOI: https://doi.org/10.1007/s00277-022-04936-2

Keywords

  • Thrombotic thrombocytopenic purpura
  • ADAMTS13
  • Pregnancy
  • Preeclampsia