Pregnancy and non-pregnancy related immune thrombotic thrombocytopenic purpura in women of reproductive age

Abstract

Pregnancy is a precipitating factor for immune thrombotic thrombocytopenic purpura (iTTP). We compared the clinical course and outcomes of iTTP in women of reproductive age, between those with pregnancy- and non-pregnancy-related iTTP. A review of all reproductive-aged women diagnosed with iTTP during 2010–2019 in seven university hospitals in Israel. Of 42 cases of iTTP, 12 (28.6%) were pregnancy-related. At presentation, the laboratory profiles did not differ significantly between those with pregnancy- and non-pregnancy-related iTTP, including hemoglobin (median 8.4 vs 8.0 g/dL), platelet count (12.5 vs. 11.5 X 109/L); and levels of bilirubin (1.23 vs. 1.82 mg/dL), lactate dehydrogenase (1615 vs. 1701 U/L), creatinine (0.61 vs. 0.79 mg/dL) and anti-ADAMTS13 antibodies titer (75 vs. 82 U/mL). The proportions of women with renal, neurologic, or hepatic involvement were similar between the groups. Cardiac involvement was more common among those with pregnancy-related disease (25.0% vs. 3.3%, P = 0.06). The median number of courses of plasma-exchange therapy was 11 for both groups. All the women were treated with parenteral corticosteroids and the rate of adjunctive treatments did not differ between the groups (P = 0.30). Four women (one-third) with pregnancy-related disease had preeclampsia. Two women (16.7%) with pregnancy-related iTTP died during the acute episode (P = 0.07); no deaths were observed in the non-pregnancy-related group. Among reproductive-aged women with iTTP, most clinical and laboratory profiles were similar between those with pregnancy- and non-pregnancy-related disease. However, the higher rates of cardiac involvement and mortality among women with pregnancy-related iTTP highlight its challenging management.

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Abbreviations

GA:

Gestational age

iTTP:

Immune thrombotic thrombocytopenia purpura

LDH:

Lactate dehydrogenase

PEX:

Plasma exchange

SGA:

Small for gestational age

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Acknowledgements

Shira Dor’s participation in this study was performed in fulfillment of the research requirements toward the MD degree. We thank Ms. Cindy Cohen for her editorial assistance.

Funding

No external funding was used in this conduct of this study.

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Authors

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All authors-study concept, design, review and approval of the final manuscript. SD, AA, AR, YK, AKP, GS, YN, ME, GS, IK, OP, ED—data acquisition. AR, YK—data analysis and writing of the manuscript.

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Correspondence to Yosef Kalish.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. This study was approved by the local institutional review board of each of the participating hospitals.

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Institutional Review Board approval waiving informed consent was obtained for this retrospective study.

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Cite this article

Rottenstreich, A., Dor, S., Keren-Politansky, A. et al. Pregnancy and non-pregnancy related immune thrombotic thrombocytopenic purpura in women of reproductive age. J Thromb Thrombolysis 51, 187–193 (2021). https://doi.org/10.1007/s11239-020-02133-4

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Keywords

  • Immune
  • Outcomes
  • Pregnancy
  • Reproductive-age
  • Thrombotic thrombocytopenic purpura