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Pregnancy-onset thrombotic thrombocytopenic purpura with nephrotic syndrome: a case report

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Abstract

Pregnancy-onset thrombotic thrombocytopenic purpura (TTP) was reported by many obstetricians and hematologists, but less by nephrologists, and the detailed clinical course of its renal complication is not known. Here, we report a case of a 33-year-old pregnant woman who suffered from pregnancy-onset TTP with nephrotic syndrome which was controlled by the termination of pregnancy. On admission, she had periorbital and lower leg edema at 32 weeks of gestation. Her serum albumin level was 2.8 g/dL and the urine protein/creatinine ratio was 4.1 g/g Cr. Besides those, she had thrombocytopenia, hemolytic anemia, and severe deficiency of A Disintegrin-like and Metalloproteinase with Thrombospondin type 1 motifs 13 (ADAMTS-13) activity. Thus, she was diagnosed with nephrotic syndrome due to pregnancy-onset TTP. A cesarean section was performed without complications for the patient and her baby. Then, all her symptoms improved shortly. She was suspected of congenital TTP because of no ADAMTS-13 inhibitor results and the persistent deficiency of ADAMTS-13 activity even after her condition improved. Pregnancy-onset TTP can cause nephrotic syndrome. Termination of pregnancy should be considered in cases with pregnancy-onset TTP to protect kidney function.

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References

  1. Sadler JE. Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura. Blood. 2008;112:11–8.

    Article  CAS  Google Scholar 

  2. Van Dorland HA, Taleghani MM, Sakai K, Friedman KD, George JN, Hrachovinova I, et al. The International Hereditary Thrombotic Thrombocytopenic Purpura Registry: key findings at enrollment until 2017. Haematologica. 2019;104:2107–15.

    Article  Google Scholar 

  3. Ferrari B, Peyvandi F. How I treat thrombotic thrombocytopenic purpura in pregnancy. Blood. 2020;136:2125–32.

    Article  CAS  Google Scholar 

  4. Moatti-Cohen M, Garrec C, Wolf M, Boisseau P, Galicier L, Azoulay E, et al. Unexpected frequency of Upshaw-Schulman syndrome in pregnancy-onset thrombotic thrombocytopenic purpura. Blood. 2012;119:5888–97.

    Article  CAS  Google Scholar 

  5. Scully M, Thomas M, Underwood M, Watson H, Langley K, Camilleri RS, et al. Thrombotic thrombocytopenic purpura and pregnancy: presentation, management, and subsequent pregnancy outcomes. Blood. 2014;124:211–9.

    Article  CAS  Google Scholar 

  6. Coppo P, Veyradier A. TTP in the setting of pregnancy: The story still has to be written. J Thromb Haemost. 2020;18:2775–7.

    Article  Google Scholar 

  7. Fujimura Y, Matsumoto M, Kokame K, Isonishi A, Soejima K, Akiyama N, et al. Pregnancy-induced thrombocytopenia and TTP, and the risk of fetal death, in Upshaw-Schulman syndrome: a series of 15 pregnancies in 9 genotyped patients. Br J Haematol. 2009;144:742–54.

    Article  CAS  Google Scholar 

  8. Edward F, Amorosi L, Ultmann John E. Thrombotic thrombocytopenic purpura report of 16 cases and review of the literature. Medicine (Baltimore). 1966;45:139–60.

    Article  Google Scholar 

  9. Epperla N, Hemauer K, Friedman KD, George JN, Foy P. Congenital thrombotic thrombocytopenic purpura related to a novel mutation in ADAMTS13 gene and management during pregnancy. Am J Hematol. 2016;91:644–6.

    Article  Google Scholar 

  10. Gupta M, Feinberg BB, Burwick RM. Thrombotic microangiopathies of pregnancy: differential diagnosis. Pregnancy Hypertens. 2018;12:29–34.

    Article  Google Scholar 

  11. Fakhouri F, Scully M, Provot F, Blasco M, Coppo P, Noris M, et al. Management of thrombotic microangiopathy in pregnancy and postpartum: report from an international working group. Blood. 2020;136:2103–17.

    Article  CAS  Google Scholar 

  12. Haller H. Thrombotic microangiopathy and the kidneys. Nephrologe. 2019;14:100–7.

    Article  Google Scholar 

  13. Scully M, Cataland S, Coppo P, de la Rubia J, Friedman KD, Kremer Hovinga J, et al. Consensus on the standardization of terminology in thrombotic thrombocytopenic purpura and related thrombotic microangiopathies. J Thromb Haemost. 2017;15:312–22.

    Article  CAS  Google Scholar 

  14. Mannucci PO, Canciani MT, Forza I, Lussana F, Lattuada A, Rossi E. Changes in health and disease of the metalloprotease that cleaves von Willebrand factor. Blood. 2001;98:2730–5.

    Article  CAS  Google Scholar 

  15. Sánchez-Luceros A, Farías CE, Amaral MM, Kempfer AC, Votta R, Marchese C, et al. von Willebrand factor-cleaving protease (ADAMTS13) activity in normal non-pregnant women, pregnant and post-delivery women. Thromb Haemost. 2004;92:1320–6.

    Article  Google Scholar 

  16. Fujimura Y, Matsumoto M, Isonishi A, Yagi H, Kokame K, Soejima K, et al. Natural history of Upshaw-Schulman syndrome based on ADAMTS13 gene analysis in Japan. J Thromb Haemost. 2011;9:283–301.

    Article  CAS  Google Scholar 

  17. Lusco MA, Fogo AB, Najafian B, Alpers CE. AJKD atlas of renal pathology: thrombotic microangiopathy. Am J Kidney Dis. 2016;68:e33–4.

    Article  Google Scholar 

  18. Kobayashi N, Ueno T, Ohashi K, Yamashita H, Takahashi Y, Sakamoto K, et al. Podocyte injury-driven intracapillary plasminogen activator inhibitor type 1 accelerates podocyte loss via uPAR-mediated β1-integrin endocytosis. Am J Physiol Ren Physiol. 2015;308:F614–26.

    Article  CAS  Google Scholar 

  19. Suzuki T, Ichikawa D, Nakata M, Watanabe S, Han W, Kohatsu K, et al. Nephrotic syndrome due to preeclampsia before 20 weeks of gestation: a case report. BMC Nephrol. 2020;21:1–6.

    Article  Google Scholar 

  20. Coric A, Resic H, Ajanovic S, Prohic N, Beciragic A. Treatment of acute kidney injury in hemolythic Uremic Syndrome (TTP). Med Arch. 2018;72:453–5.

    Article  Google Scholar 

  21. Manea M, Kristoffersson A, Schneppenheim R, Saleem MA, Mathieson PW, Mörgelin M, et al. Podocytes express ADAMTS13 in normal renal cortex and in patients with thrombotic thrombocytopenic purpura. Br J Haematol. 2007;138:651–62.

    Article  CAS  Google Scholar 

  22. Garg P. A review of podocyte biology. Am J Nephrol. 2018;47:3–13.

    Article  CAS  Google Scholar 

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Acknowledgements

We thank the patient and all involved medical staffs.

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Authors and Affiliations

  1. Department of Nephrology, Japanese Red Cross Medical Center, 4-1-22 Hiro-o, Shibuya-Ku, Tokyo, 150-8935, Japan

    Ryunosuke Noda & Yoshitaka Ishibashi

  2. Department of Obstetrics, Japanese Red Cross Medical Center, 4-1-22 Hiro-o, Shibuya-Ku, Tokyo, 150-8935, Japan

    Yuki Kakinuma, Kensuke Suzuki, Sanae Ide & Akito Miyauchi

  3. Department of Pathology, Japanese Red Cross Medical Center, 4-1-22 Hiro-o, Shibuya-Ku, Tokyo, 150-8935, Japan

    Yuan Bae

Authors
  1. Ryunosuke Noda
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  2. Yuki Kakinuma
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  3. Kensuke Suzuki
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  4. Sanae Ide
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  5. Yuan Bae
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  6. Akito Miyauchi
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  7. Yoshitaka Ishibashi
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Correspondence to Ryunosuke Noda.

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We performed all of the treatment and the examination in accordance with the guidelines laid down in the Declaration of Helsinki.

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Informed consent for the treatment and the renal biopsy was obtained from the patient.

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Noda, R., Kakinuma, Y., Suzuki, K. et al. Pregnancy-onset thrombotic thrombocytopenic purpura with nephrotic syndrome: a case report. CEN Case Rep 11, 203–207 (2022). https://doi.org/10.1007/s13730-021-00654-2

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  • DOI: https://doi.org/10.1007/s13730-021-00654-2

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