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Immune Thrombocytopenia and Microangiopathies in Pregnancy

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Obstetric Catastrophes

Abstract

Immune thrombocytopenic purpura (ITP) is an acquired disease that raises diagnostic and therapeutic doubts in the clinician for being a diagnosis of exclusion, fear of bleeding, and not being able to correct the event with simple transfusion measures. Usually 7–10% of pregnant women will have thrombocytopenia; ITP is the second most frequent cause of thrombocytopenia in pregnant women after gestational thrombocytopenia, representing 1–4% of the causes of thrombocytopenia. ITP in pregnant women has one case incidence per 10,000 to 100,000 women, but in the general adult population, the incidence of ITP is 2.64 in 100,000 people, and the average age of presentation is 56 years. The mainstay of treatment is based on avoiding bleeding, and this is achieved with a multidisciplinary management: the obstetrician, anesthesiologist, hematologist, and neonatologist. And although only one third of the patients will require treatment, we must consider the platelet value, if there are previous stigmas of bleeding, if there is any associated coagulopathy, and mode of delivery as well as use of epidural and neonate safety. Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are thrombotic microangiopathies, both acute, rare life-threatening conditions, and the biggest challenge is timely and rapid diagnosis.

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References

  1. Gernsheimer T, James AH, Stasi R. How I treat thrombocytopenia in pregnancy. Blood. 2013;121(1):38–47. https://doi.org/10.1182/blood-2012-08-448944. Epub 2012/11/13. PubMed PMID: 23149846

    Article  CAS  PubMed  Google Scholar 

  2. Rajasekhar A, Gernsheimer T, Roberto S, James AH. 2013 Clinical practice guide on thrombocytopenia in pregnancy. In: Hematology So, editor. American Society of Hematology; 2013.

    Google Scholar 

  3. Alonso MAnS, García VV. Directrices de diagnóstico, tratamiento y seguimiento de la PTI: Documento de Consenso. In: López A, editor. 2011.

    Google Scholar 

  4. Frederiksen H, Schmidt K. The incidence of idiopathic thrombocytopenic purpura in adults increases with age. Blood. 1999;94(3):909–13. Epub 1999/07/27. PubMed PMID: 10419881

    Article  CAS  PubMed  Google Scholar 

  5. Neunert C, Lim W, Crowther M, Cohen A, Solberg L Jr, Crowther MA, et al. The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia. Blood. 2011;117(16):4190–207. https://doi.org/10.1182/blood-2010-08-302984. Epub 2011/02/18. PubMed PMID: 21325604

    Article  CAS  PubMed  Google Scholar 

  6. Vainchenker W, Raslova H. Megakaryocyte polyploidization: role in platelet production. Platelets. 2019:1–10. https://doi.org/10.1080/09537104.2019.1667497. Epub 2019/09/24. PubMed PMID: 31544577

  7. Kaushansky K. Historical review: megakaryopoiesis and thrombopoiesis. Blood. 2008;111(3):981–6. https://doi.org/10.1182/blood-2007-05-088500. Epub 2008/01/29. PubMed PMID: 18223171; PubMed Central PMCID: PMCPMC2214745

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Hirokawa M, Fujishima N, Togashi M, Saga A, Omokawa A, Saga T, et al. High-throughput sequencing of IgG B-cell receptors reveals frequent usage of the rearranged IGHV4-28/IGHJ4 gene in primary immune thrombocytopenia. Sci Rep. 2019;9(1):8645. https://doi.org/10.1038/s41598-019-45264-2. Epub 2019/06/16. PubMed PMID: 31201346; PubMed Central PMCID: PMCPMC6570656

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Zufferey A, Kapur R, Semple JW. Pathogenesis and therapeutic mechanisms in Immune Thrombocytopenia (ITP). J Clin Med. 2017;6(2) https://doi.org/10.3390/jcm6020016. Epub 2017/02/18. PubMed PMID: 28208757; PubMed Central PMCID: PMCPMC5332920

  10. Rosa Maria RN, Laura RL, Angeles PB, Laura LB. Use of Romiplostim during pregnancy as a rescue therapy in primary immune thrombocytopenia: literature review and case description. Platelets. 2019:1–4. https://doi.org/10.1080/09537104.2019.1615613. Epub 2019/05/23. PubMed PMID: 31116059

  11. Gil WR. Diagnosis and treatment of immune thrombocytopenic purpura. Rev Med Hered. 2015;26:246–55.

    Google Scholar 

  12. Rodeghiero F, Stasi R, Gernsheimer T, Michel M, Provan D, Arnold DM, et al. Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group. Blood. 2009;113(11):2386–93. https://doi.org/10.1182/blood-2008-07-162503. Epub 2008/11/14. PubMed PMID: 19005182

    Article  CAS  PubMed  Google Scholar 

  13. Kistangari G, McCrae KR. Immune thrombocytopenia. Hematol Oncol Clin North Am. 2013;27(3):495–520. https://doi.org/10.1016/j.hoc.2013.03.001. Epub 2013/05/30. PubMed PMID: 23714309; PubMed Central PMCID: PMCPMC3672858

    Article  PubMed  Google Scholar 

  14. Páramo Fernández JA, Alfonso Piérola A, Varea Díaz S. Alteraciones de la hemostasia primaria. Púrpuras y alteraciones de las plaquetas. Medicine – Programa de Formación Médica Continuada Acreditado. 2012;11(22):337–1344. https://doi.org/10.1016/s0304-5412(12)70460-2.

    Article  Google Scholar 

  15. Jang JH, Kim JY, Mun YC, Bang SM, Lim YJ, Shin DY, et al. Management of immune thrombocytopenia: Korean experts recommendation in 2017. Blood Res. 2017;52(4):254–63. https://doi.org/10.5045/br.2017.52.4.254. Epub 2018/01/16. PubMed PMID: 29333401; PubMed Central PMCID: PMCPMC5762735

    Article  PubMed  PubMed Central  Google Scholar 

  16. Gill KK, Kelton JG. Management of idiopathic thrombocytopenic purpura in pregnancy. Semin Hematol. 2000;37(3):275–89. Epub 2000/08/15. PubMed PMID: 10942222

    Article  CAS  PubMed  Google Scholar 

  17. Khellaf M, Loustau V, Bierling P, Michel M, Godeau B. Thrombocytopenia and pregnancy. Rev Med Interne. 2012;33(8):446–52. https://doi.org/10.1016/j.revmed.2012.05.011. Epub 2012/06/30. PubMed PMID: 22742709

    Article  CAS  PubMed  Google Scholar 

  18. Payne YC, González DC. Thrombocytopenia in pregnancy: gestational, immune and congenital Revista Cubana de Hematol. Inmunol y Hemoter. 2014;30(3):196–207.

    Google Scholar 

  19. Yu XJ, Liang MF, Zhang SY, Liu Y, Li JD, Sun YL, et al. Fever with thrombocytopenia associated with a novel bunyavirus in China. N Engl J Med. 2011;364(16):1523–32. https://doi.org/10.1056/NEJMoa1010095. Epub 2011/03/18. PubMed PMID: 21410387; PubMed Central PMCID: PMCPMC3113718

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  20. Cines DB, Bussel JB, Liebman HA, Luning Prak ET. The ITP syndrome: pathogenic and clinical diversity. Blood. 2009;113(26):6511–21. https://doi.org/10.1182/blood-2009-01-129155. Epub 2009/04/28. PubMed PMID: 19395674; PubMed Central PMCID: PMCPMC2710913

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Goldman BG, Hehir MP, Yambasu S, O’Donnell EM. The presentation and management of platelet disorders in pregnancy. Eur J Haematol. 2018;100(6):560–6. https://doi.org/10.1111/ejh.13049. Epub 2018/02/22. PubMed PMID: 29464836

    Article  PubMed  Google Scholar 

  22. Provan D, Stasi R, Newland AC, Blanchette VS, Bolton-Maggs P, Bussel JB, et al. International consensus report on the investigation and management of primary immune thrombocytopenia. Blood. 2010;115(2):168–86. https://doi.org/10.1182/blood-2009-06-225565. Epub 2009/10/23. PubMed PMID: 19846889

    Article  CAS  PubMed  Google Scholar 

  23. Hematology ASo, editor. Self-assessment program seventh edition. American Society of Hematology; 2019.

    Google Scholar 

  24. Amorosi EL, Ultmann JE. Thrombotic thrombocytopenic purpura: report of 16 cases and review of the literature. Medicine. 1966;45(2):139–60.

    Article  Google Scholar 

  25. von Auer C, von Krogh AS, Kremer Hovinga JA, Lammle B. Current insights into thrombotic microangiopathies: Thrombotic thrombocytopenic purpura and pregnancy. Thromb Res. 2015;135(Suppl 1):S30–3. https://doi.org/10.1016/S0049-3848(15)50437-4. Epub 2015/04/24. PubMed PMID: 25903530

    Article  CAS  Google Scholar 

  26. Scully M, Yarranton H, Liesner R, Cavenagh J, Hunt B, Benjamin S, et al. Regional UK TTP registry: correlation with laboratory ADAMTS 13 analysis and clinical features. Br J Haematol. 2008;142(5):819–26. https://doi.org/10.1111/j.1365-2141.2008.07276.x. Epub 2008/07/22. PubMed PMID: 18637802

    Article  PubMed  Google Scholar 

  27. Scully M, Hunt BJ, Benjamin S, Liesner R, Rose P, Peyvandi F, et al. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol. 2012;158(3):323–35. https://doi.org/10.1111/j.1365-2141.2012.09167.x. Epub 2012/05/26. PubMed PMID: 22624596

    Article  PubMed  Google Scholar 

  28. Mariotte E, Azoulay E, Galicier L, Rondeau E, Zouiti F, Boisseau P, et al. Epidemiology and pathophysiology of adulthood-onset thrombotic microangiopathy with severe ADAMTS13 deficiency (thrombotic thrombocytopenic purpura): a cross-sectional analysis of the French national registry for thrombotic microangiopathy. Lancet Haematol. 2016;3(5):e237–45. https://doi.org/10.1016/S2352-3026(16)30018-7. Epub 2016/05/03. PubMed PMID: 27132698

    Article  PubMed  Google Scholar 

  29. Marchi R, Rojas H. Effect of von Willebrand factor on clot structure and lysis. Blood Coagul Fibrinolysis. 2015;26(5):533–6. https://doi.org/10.1097/MBC.0000000000000284. Epub 2015/03/27. PubMed PMID: 25811448

    Article  CAS  PubMed  Google Scholar 

  30. Beranger N, Benghezal S, Savigny S, Capdenat S, Joly BS, Coppo P, et al. Loss of von Willebrand factor high-molecular-weight multimers at acute phase is associated with detectable anti-ADAMTS13 IgG and neurological symptoms in acquired thrombotic thrombocytopenic purpura. Thromb Res. 2019;181:29–35. https://doi.org/10.1016/j.thromres.2019.07.012. Epub 2019/07/23. PubMed PMID: 31330376

    Article  CAS  PubMed  Google Scholar 

  31. 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(24):5888–97. https://doi.org/10.1182/blood-2012-02-408914. Epub 2012/05/02. PubMed PMID: 22547583

    Article  CAS  PubMed  Google Scholar 

  32. van Dorland HA, Mansouri Taleghani M, Sakai K, Friedman KD, George JN, Hrachovinova I, et al. The International Hereditary Thrombotic Thrombocytopenic Purpura Registry: Key findings at enrolment until 2017. Haematologica. 2019; https://doi.org/10.3324/haematol.2019.216796. Epub 2019/02/23. PubMed PMID: 30792199

  33. La Rubia JD, Pérez F, Navarro A. Síndrome HELLP. Med Clin. 2001;117(2):64–8.

    Article  Google Scholar 

  34. Lattuada A, Rossi E, Calzarossa C, Candolfi R, Mannucci PM. Mild to moderate reduction of a von Willebrand factor cleaving protease (ADAMTS-13) in pregnant women with HELLP microangiopathic syndrome. Haematologica. 2003;88(9):1029–34. Epub 2003/09/13. PubMed PMID: 12969811

    CAS  PubMed  Google Scholar 

  35. Page EE, Kremer Hovinga JA, Terrell DR, Vesely SK, George JN. Thrombotic thrombocytopenic purpura: diagnostic criteria, clinical features, and long-term outcomes from 1995 through 2015. Blood Adv. 2017;1(10):590–600. https://doi.org/10.1182/bloodadvances.2017005124. Epub 2018/01/04. PubMed PMID: 29296701; PubMed Central PMCID: PMCPMC5728353 interests

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  36. Chiasakul T, Cuker A. Clinical and laboratory diagnosis of TTP: an integrated approach. Hematology Am Soc Hematol Educ Program. 2018;2018(1):530–8. https://doi.org/10.1182/asheducation-2018.1.530. Epub 2018/12/07. PubMed PMID: 30504354; PubMed Central PMCID: PMCPMC6246034 Bayer, Bioverativ, Novo Nordisk, Pfizer, Shire, Spark, and Syntimmune and has consulted for Genzyme, Kedrion, and Synergy. T.C. declares no competing financial interests

    Article  PubMed  PubMed Central  Google Scholar 

  37. Bommer M, Wolfle-Guter M, Bohl S, Kuchenbauer F. The differential diagnosis and treatment of thrombotic microangiopathies. Dtsch Arztebl Int. 2018;115(19):327–34. https://doi.org/10.3238/arztebl.2018.0327. Epub 2018/06/08. PubMed PMID: 29875054; PubMed Central PMCID: PMCPMC5997890

    Article  PubMed  PubMed Central  Google Scholar 

  38. Scully M, Goodship T. How I treat thrombotic thrombocytopenic purpura and atypical haemolytic uraemic syndrome. Br J Haematol. 2014;164(6):759–66. https://doi.org/10.1111/bjh.12718. Epub 2014/01/07. PubMed PMID: 24387053; PubMed Central PMCID: PMCPMC4163720

    Article  PubMed  PubMed Central  Google Scholar 

  39. Rock GA, Shumak KH, Buskard NA, Blanchette VS, Kelton JG, Nair RC, et al. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group. N Engl J Med. 1991;325(6):393–7. https://doi.org/10.1056/NEJM199108083250604. Epub 1991/08/08. PubMed PMID: 2062330

    Article  CAS  PubMed  Google Scholar 

  40. Schwartz J, Padmanabhan A, Aqui N, Balogun RA, Connelly-Smith L, Delaney M, et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice-Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Seventh Special Issue. J Clin Apher. 2016;31(3):149–62. https://doi.org/10.1002/jca.21470. Epub 2016/06/21. PubMed PMID: 27322218

    Article  PubMed  Google Scholar 

  41. Scully M, McDonald V, Cavenagh J, Hunt BJ, Longair I, Cohen H, et al. A phase 2 study of the safety and efficacy of rituximab with plasma exchange in acute acquired thrombotic thrombocytopenic purpura. Blood. 2011;118(7):1746–53. https://doi.org/10.1182/blood-2011-03-341131. Epub 2011/06/04. PubMed PMID: 21636861

    Article  CAS  PubMed  Google Scholar 

  42. Scully M, Knobl P, Kentouche K, Rice L, Windyga J, Schneppenheim R, et al. Recombinant ADAMTS-13: first-in-human pharmacokinetics and safety in congenital thrombotic thrombocytopenic purpura. Blood. 2017;130(19):2055–63. https://doi.org/10.1182/blood-2017-06-788026. Epub 2017/09/16. PubMed PMID: 28912376; PubMed Central PMCID: PMCPMC5680611

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  43. 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(2):211–9. https://doi.org/10.1182/blood-2014-02-553131. Epub 2014/05/27. PubMed PMID: 24859360

    Article  CAS  PubMed  Google Scholar 

  44. Jokiranta TS. HUS and atypical HUS. Blood. 2017;129(21):2847–56. https://doi.org/10.1182/blood-2016-11-709865. Epub 2017/04/19. PubMed PMID: 28416508; PubMed Central PMCID: PMCPMC5445567

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  45. Loirat C, Fremeaux-Bacchi V. Atypical hemolytic uremic syndrome. Orphanet J Rare Dis. 2011;6:–60. https://doi.org/10.1186/1750-1172-6-60. Epub 2011/09/10. PubMed PMID: 21902819; PubMed Central PMCID: PMCPMC3198674

  46. S M. Thrombocytopenia in pregnancy. In: Clínica Universitaria Reina Fabiola CC, editor. XIII Congreso del Grupo CAHT; Septiembre 20182018. p. 117–24.

    Google Scholar 

  47. Karpman D, Loos S, Tati R, Arvidsson I. Haemolytic uraemic syndrome. J Intern Med. 2017;281(2):123–48. https://doi.org/10.1111/joim.12546. Epub 2016/10/11. PubMed PMID: 27723152

    Article  PubMed  Google Scholar 

  48. Rodriguez de Cordoba S, Hidalgo MS, Pinto S, Tortajada A. Genetics of atypical hemolytic uremic syndrome (aHUS). Semin Thromb Hemost. 2014;40(4):422–30. https://doi.org/10.1055/s-0034-1375296. Epub 2014/05/07. PubMed PMID: 24799305

    Article  CAS  PubMed  Google Scholar 

  49. Michael M, Elliott EJ, Craig JC, Ridley G, Hodson EM. Interventions for hemolytic uremic syndrome and thrombotic thrombocytopenic purpura: a systematic review of randomized controlled trials. Am J Kidney Dis. 2009;53(2):259–72. https://doi.org/10.1053/j.ajkd.2008.07.038. Epub 2008/10/28. PubMed PMID: 18950913

    Article  PubMed  Google Scholar 

  50. Sarno L, Tufano A, Maruotti GM, Martinelli P, Balletta MM, Russo D. Eculizumab in pregnancy: a narrative overview. J Nephrol. 2019;32(1):17–25. https://doi.org/10.1007/s40620-018-0517-z. Epub 2018/08/31. PubMed PMID: 30159857

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Centro de Neurociencias y Unidad de Investigacion Clínica, Instituto de Investigaciones Científicas y Servicios de Alta Tecnología (INDICASAT AIP), Panama City, Republic of Panama

    Alcibiades E. Villarreal

  2. Department of Hematology, Complejo Hospitalario Dr. Arnulfo Arias Madrid de la Caja de Seguro Social, Panama City, Republic of Panama

    Lineth López

Authors
  1. Alcibiades E. Villarreal
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Correspondence to Lineth López .

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Editors and Affiliations

  1. Critical Care Obstetrics Unit, Hospital Doctor Arnulfo Arias Madrid, Panama City, Panama

    Carlos Montufar

  2. Division of Critical Care, Karl Heusner Memorial Hospital, Belize City, Belize

    Jorge Hidalgo

  3. Houston Center for Maternal-Fetal Medicine, Houston, TX, USA

    Alfredo F. Gei

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Villarreal, A.E., López, L. (2021). Immune Thrombocytopenia and Microangiopathies in Pregnancy. In: Montufar, C., Hidalgo, J., Gei, A.F. (eds) Obstetric Catastrophes. Springer, Cham. https://doi.org/10.1007/978-3-030-70034-8_45

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