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The course of acquired von Willebrand syndrome during pregnancy among patients with essential thrombocytosis

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Abstract

To investigate the course of acquired type 2A von Willebrand syndrome (AVWS) in relation to patient management and outcomes among pregnant patients with essential thrombocytosis (ET). A review of pregnant women with ET evaluated for AVWS at the beginning of pregnancy and at the third trimester. Eighteen women with 24 pregnancies were included in this study. A history of bleeding was noted in 8 (44%) patients. In 20 (83%) pregnancies AVWS was evident at the initial testing. Following initial testing, antithrombotic therapy was administered in 22 (92%) pregnancies (aspirin, n = 20 and low-molecular-weight heparin, n = 2). In the remaining two pregnancies, VWF:RCo levels were below 30%; thus, aspirin was given only after repeat testing at 14–16 weeks. At third trimester testing, median VWF:RCo levels were significantly higher than at the initial testing (86 vs. 48%, P < 0.001), with no evidence of AVWS in any of the patients. Significant increases were also observed in the VWF:Ag level (127 vs. 84%, P < 0.001), the VWF:RCo/VWF:Ag ratio (0.75 vs. 0.54, P < 0.001) and the FVIII level (103 vs. 68%, P < 0.001); while platelet count (359 vs. 701 × 109/l, P < 0.001) and hemoglobin level (11.6 vs. 13.4 g/dl, P < 0.001) decreased. Neuraxial anesthesia was safely performed in 17 (71%) pregnancies. No significant bleeding events occurred during pregnancy and delivery. AVWS-related abnormalities in women with ET mostly improved during pregnancy, with favorable maternal and fetal outcomes. VWF parameters should be tested at early pregnancy and repeated at the third trimester, to guide pregnancy and delivery management.

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Abbreviations

AVWS:

Acquired von Willebrand syndrome

ET:

Essential thrombocytosis

VWD:

Von Willebrand disease

VWF:

Von Willebrand factor

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Acknowledgements

We would like to thank Ms. Cindy Cohen for her editorial assistance.

Author information

Authors and Affiliations

  1. Department of Obstetrics and Gynecology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel

    Amihai Rottenstreich & Hagai Amsalem

  2. Braun School of Public Health and Community Medicine, Faculty of Medicine of the Hebrew University and Hadassah, Jerusalem, Israel

    Geffen Kleinstern

  3. Department of Health Sciences Research, Mayo Clinic, Rochester, MN, USA

    Geffen Kleinstern

  4. Department of Hematology, Hadassah-Hebrew University Medical Center, POB 12000, 91120, Jerusalem, Israel

    Yosef Kalish

Authors
  1. Amihai Rottenstreich
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  2. Geffen Kleinstern
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  3. Hagai Amsalem
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  4. Yosef Kalish
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Contributions

AR and YK drafted the initial manuscript, reviewed and revised the manuscript. GK performed the statistical analyses for this study. HA, treated the patients and reviewed and revised the manuscript. All authors read and approved the final manuscript as submitted.

Corresponding author

Correspondence to Yosef Kalish.

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Conflict of interest

The authors declare that they have no conflicts of interest.

Ethical approval

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 Hadassah Medical Center Helsinki Committee.

Informed consent

Institutional review board approval waiving informed consent was obtained for this retrospective study.

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Rottenstreich, A., Kleinstern, G., Amsalem, H. et al. The course of acquired von Willebrand syndrome during pregnancy among patients with essential thrombocytosis. J Thromb Thrombolysis 46, 304–309 (2018). https://doi.org/10.1007/s11239-018-1663-9

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  • DOI: https://doi.org/10.1007/s11239-018-1663-9

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