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Thalassemia

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Consults in Obstetric Anesthesiology

Abstract

Normal adult hemoglobin consists of two alpha and two beta globin chains, and disorders of either of these subunits cause alpha- or beta-thalassemia, respectively. Mutations and deletions in the polypeptide chains of hemoglobin result in the defective synthesis of hemoglobin seen in hemophilia, ranging from complete absence to reduced production of polypeptide chains. Cardiovascular, pulmonary, hematologic, renal, and endocrine compromise may occur during pregnancy. Pregnancy-related complications have been reported, and anesthetic challenges may occur due to multisystem involvement and to bony abnormalities.

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References

  1. Leung TY, Lao TT. Thalassaemia in pregnancy. Best Pract Res Clin Obstet Gynaecol. 2012;26(1):37–51.

    Article  Google Scholar 

  2. Higgs DR, Engel JD, Stamatoyannopoulos G. Thalassaemia. Lancet. 2012;379(9813):373–83.

    Article  CAS  Google Scholar 

  3. Petrakos G, Andriopoulos P, Tsironi M. Pregnancy in women with thalassemia: challenges and solutions. Int J Womens Health. 2016;8:441–51.

    Article  CAS  Google Scholar 

  4. Saliba AN, Taher AT. Morbidities in non-transfusion-dependent thalassemia. Ann N Y Acad Sci. 2016;1368(1):82–94.

    Article  CAS  Google Scholar 

  5. Staikou C, Stavroulakis E, Karmaniolou I. A narrative review of peri-operative management of patients with thalassaemia. Anaesthesia. 2014;69(5):494–510.

    Article  CAS  Google Scholar 

  6. Bezirgiannidou Z, Christoforidou A, Kontekaki E, Anastasiadis AG, Papamichos SI, Menexidou H, et al. Hyperhemolytic syndrome complicating a delayed hemolytic transfusion reaction due to anti-P1 Alloimmunization, in a pregnant woman with HbO-Arab/β-thalassemia. Mediterr J Hematol Infect Dis. 2016;8(1):e2016053.

    Article  Google Scholar 

  7. Shimada Y, Hidaka N, Maehara K, Murata M, Fujita Y, Kato K. Pulmonary thromboembolism during pregnancy in a woman with β-thalassaemia who had undergone splenectomy. J Obstet Gynaecol (Lahore). 2016;36(7):1–2.

    Google Scholar 

  8. Thompson AA, Kim H-Y, Singer ST, Vichinsky E, Eile J, Yamashita R, et al. Pregnancy outcomes in women with thalassemia in North America and the United Kingdom. Am J Hematol. 2013;88(9):771–3.

    Article  Google Scholar 

  9. Mancuso A, Giacobbe A, De Vivo A, Ardita FV, Meo A. Pregnancy in patients with Beta-Thalassaemia major: maternal and Foetal outcome. Acta Haematol. 2008;119(1):15–7.

    Article  Google Scholar 

  10. Okby R, Mazor M, Erez O, Beer-Weizel R, Hershkovitz R. Reversal of mirror syndrome after selective feticide of a hydropic fetus in a dichorionic diamniotic twin pregnancy. J Ultrasound Med. 2015;34(2):351–3.

    Article  Google Scholar 

  11. Zhao Y, Liu G, Wang J, Yang J, Shen D, Zhang X. Mirror syndrome in a Chinese hospital: diverse causes and maternal fetal features. J Matern Neonatal Med. 2013;26(3):254–8.

    Article  Google Scholar 

  12. Fa G, Sg V, Fauzia M, Cianci S, Pafumi C, Palumbo MA. Beta-thalassemia major and pregnancy. Bratisl Lek Listy. 2013;114(9):523–5.

    Google Scholar 

  13. Voskaridou E, Balassopoulou A, Boutou E, Komninaka V, Christoulas D, Dimopoulou M, et al. Pregnancy in beta-thalassemia intermedia: 20-year experience of a Greek thalassemia center. Eur J Haematol. 2014;93(6):492–9.

    Article  Google Scholar 

  14. Charoenboon C, Jatavan P, Traisrisilp K, Tongsong T. Pregnancy outcomes among women with beta-thalassemia trait. Arch Gynecol Obstet. 2016;293(4):771–4.

    Article  CAS  Google Scholar 

  15. Vichinsky EP. Clinical manifestations of α-thalassemia. Cold Spring Harb Perspect Med. 2013;3(5):a011742.

    Article  Google Scholar 

  16. Saliba AN, El Rassi F, Taher AT. Clinical monitoring and management of complications related to chelation therapy in patients with β-thalassemia. Expert Rev Hematol. 2016;9(2):151–68.

    Article  CAS  Google Scholar 

  17. Chou ST, Liem RI, Thompson AA. Challenges of alloimmunization in patients with haemoglobinopathies. Br J Haematol. 2012;159(4):394–404.

    Article  CAS  Google Scholar 

  18. Gray G, Nelson-Piercy C. Thromboembolic disorders in obstetrics. Best Pract Res Clin Obstet Gynaecol. 2012;26:53–64.

    Article  Google Scholar 

  19. Singounas EG, Sakas DE, Hadley DM, Chalevelakis G, Sfakianos G, Raptis S, et al. Paraplegia in a pregnant thalassemic woman due to extramedullary hematopoiesis: successful management with transfusions. Surg Neurol. 1991;36(3):210–5.

    Article  CAS  Google Scholar 

  20. Orr D. Difficult intubation: a hazard in thalassaemia. A case report. Br J Anaesth. 1967;39(7):585–6.

    Article  CAS  Google Scholar 

  21. Ali S, Khan FA. Anaesthetic management of two patients with beta-thalassaemia intermedia. J Pak Med Assoc. 2010;60(7):582–4.

    PubMed  Google Scholar 

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Correspondence to Xiwen Zheng M.D. .

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Zheng, X., Mankowitz, S.K.W. (2018). Thalassemia. In: Mankowitz, S. (eds) Consults in Obstetric Anesthesiology. Springer, Cham. https://doi.org/10.1007/978-3-319-59680-8_158

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  • DOI: https://doi.org/10.1007/978-3-319-59680-8_158

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-59679-2

  • Online ISBN: 978-3-319-59680-8

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