Home A case of post-splenectomy transfusion-dependent homozygous beta-thalassemia major complicated with myocardial siderosis and osteoporosis and usage of iron-chelating therapy with deferiprone in pregnancy
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A case of post-splenectomy transfusion-dependent homozygous beta-thalassemia major complicated with myocardial siderosis and osteoporosis and usage of iron-chelating therapy with deferiprone in pregnancy

  • Mohd Faizal Ahmad EMAIL logo , Nur Zawani Zakaria , Noorazizah Arsad , Kah Teik Chew , Muhammad Azrai Abu ORCID logo , Mohd Nasir Shafiee and Mohd Hashim Omar
Published/Copyright: July 13, 2019

Abstract

Beta-thalassemia major is a subtype component of hemoglobinopathies; autosomal recessive disorders complicated with anemia that affect at least 50,000 babies each year. It contributes to problems in reproductive entities such as infertility due to iron deposition in the endocrine organs, which leads to malfunction of the hypothalamus-pituitary axis. Due to this, there have been very few pregnancies discovered and reported with this type of condition as they usually required an ovulation-induction agent with assisted reproductive technique to achieved pregnancy. We report a successful spontaneous pregnancy in a woman with beta-thalassemia major who underwent splenectomy with lifelong transfusion-dependence complicated with myocardial siderosis and osteoporosis. The close monitoring and regular blood transfusion are a core of successful support to this type of pregnancy. The unintentional consumption of Fosamax, hydroxyurea and deferiprone (Ferriprox) up till 20 weeks of gestation did not show any adverse effects on fetal well-being. As expected, this pregnancy ended with the preterm delivery via cesarean section due to intrauterine growth restriction with oligohydramnios, and currently, this child is thriving. We concluded that pregnancy is not a contraindication in beta-thalassemia major; complex individual care is needed to achieve a safe outcome for the mother.

Author statement

  1. Research funding: Authors state no funding involved.

  2. Conflict of interest: Authors state no conflict of interest.

  3. Informed consent: Informed consent was taken and approved.

  4. Ethical approval: This case report was approved for publication based on ethical regulations. Consent from the patient was obtained and the case is allowed for publication solely for educational purposes.

References

[1] Oliver NF, Nathan DG, MacMillan JH, Wayne AS, Liu PP, McGee A, et al. Survival in medically treated patients with homozygous beta thalassemia. N Engl J Med. 1994;331:574–8.10.1056/NEJM199409013310903Search in Google Scholar PubMed

[2] Aydinok Y, Darcan S, Polat A, Kavakli K, Nigli G, Coker M, et al. Endocrine complications in patients with beta thalassemia major. J Trop Pediatr. 2002;48:50–4.10.1093/tropej/48.1.50Search in Google Scholar PubMed

[3] Bronspiegel-Weintrob N, Oliveri NF, Tyler B, Andrews DF, Freedman MH, Holland FJ. Effect of age at the start of iron chelation therapy on gonadal function in beta thalassemia major. N Engl J Med. 1990;323:713–9.10.1056/NEJM199009133231104Search in Google Scholar PubMed

[4] Ryan K, Bain BJ, Worthington D, James J, Plews D, Mason A, et al. British Committee for Standards in Haemotology. Significant haemoglobinopathies: guidelines for screening and diagnosis. Br J Haematol. 2010;149:35–49.10.1111/j.1365-2141.2009.08054.xSearch in Google Scholar

[5] Chatterjee R, Katz M, Cox TF, Porter JB. Prospective study of the hypothalamic-pituitary axis in thalassaemic patients who developed secondary amenorrhoea. Clin Endocrinol (Oxf). 1993;39:287–96.10.1111/j.1365-2265.1993.tb02368.xSearch in Google Scholar PubMed

[6] Diamantidis MD, Neokleous N, Agapidou A, Vetsiou E, Manafas A, Fotiou P, et al. Iron chelation therapy of transfusion-dependent beta-thalassemia during pregnancy in the era of novel drugs: is deferasirox toxic? Int J Hematol. 2011;103:537–44.10.1007/s12185-016-1945-ySearch in Google Scholar

[7] Origa R, Piga A, Quarta G, Forni GL, Longo F, Melpignano A, et al. Pregnancy and β-thalassemia: an Italian multicenter experience. Haematologica. 2010;95:376–81.10.3324/haematol.2009.012393Search in Google Scholar PubMed

[8] Tampakoudis P, Tsatalas C, Mamopoulos M, Tantanassis T, Christakis JI, Sinakos Z, et al. Transfusion dependent homozygous beta-thalassaemia major: successful pregnancy in five cases. Eur J Obstet Gynecol Reprod Biol. 1997;74:127–31.10.1016/S0301-2115(97)00089-4Search in Google Scholar PubMed

[9] Modell B, Khan M, Darlison M, Westwood MA, Ingram D, Pennell DJ. Improved survival of thalassaemia major in the UK and relation to T2* cardiovascular magnetic resonance. J Cardiovasc Magn Reson. 2008;10:42.10.1186/1532-429X-10-42Search in Google Scholar PubMed PubMed Central

Received: 2019-02-23
Accepted: 2019-04-01
Published Online: 2019-07-13

©2019 Walter de Gruyter GmbH, Berlin/Boston

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