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, Nur Zawani Zakaria, Noorazizah Arsad, Chew Kah Teik, Muhammad Azrai Abu, Mohamad Nasir Shafiee, Mohd Hashim Omar

Research output: Contribution to journalArticle

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.

Original languageEnglish
JournalHormone Molecular Biology and Clinical Investigation
DOIs
Publication statusPublished - 1 Jan 2019

Fingerprint

Siderosis
beta-Thalassemia
Splenectomy
Osteoporosis
Iron
Pregnancy
Therapeutics
Oligohydramnios
Alendronate
Hemoglobinopathies
Assisted Reproductive Techniques
Hydroxyurea
Ovulation Induction
deferiprone
Cesarean Section
Blood Transfusion
Infertility
Hypothalamus
Anemia
Mothers

Keywords

  • beta-thalassemia major
  • hemoglobinopathies
  • pregnancy
  • preterm delivery

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Molecular Biology
  • Endocrinology

Cite this

@article{3a42702ba6f0478db5e6edf46dfcbba9,
title = "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",
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.",
keywords = "beta-thalassemia major, hemoglobinopathies, pregnancy, preterm delivery",
author = "Ahmad, {Mohd Faizal} and Zakaria, {Nur Zawani} and Noorazizah Arsad and {Kah Teik}, Chew and Abu, {Muhammad Azrai} and Shafiee, {Mohamad Nasir} and Omar, {Mohd Hashim}",
year = "2019",
month = "1",
day = "1",
doi = "10.1515/hmbci-2019-0005",
language = "English",
journal = "Hormone Molecular Biology and Clinical Investigation",
issn = "1868-1883",
publisher = "Walter de Gruyter GmbH",

}

TY - JOUR

T1 - 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

AU - Ahmad, Mohd Faizal

AU - Zakaria, Nur Zawani

AU - Arsad, Noorazizah

AU - Kah Teik, Chew

AU - Abu, Muhammad Azrai

AU - Shafiee, Mohamad Nasir

AU - Omar, Mohd Hashim

PY - 2019/1/1

Y1 - 2019/1/1

N2 - 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.

AB - 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.

KW - beta-thalassemia major

KW - hemoglobinopathies

KW - pregnancy

KW - preterm delivery

UR - http://www.scopus.com/inward/record.url?scp=85069693554&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85069693554&partnerID=8YFLogxK

U2 - 10.1515/hmbci-2019-0005

DO - 10.1515/hmbci-2019-0005

M3 - Article

JO - Hormone Molecular Biology and Clinical Investigation

JF - Hormone Molecular Biology and Clinical Investigation

SN - 1868-1883

ER -