The Effect of Prolonged Antenatal Intravenous Immunoglobulin Treatment in Preventing Gestational Alloimmune Liver Disease—A Case Series with Literature Review

Abstract

Background Gestational alloimmune liver disease (GALD) is characterized by maternal IgG-directed fetal hepatocyte damage and can lead to severe liver failure and fetal or infant death. Moreover, GALD is associated with a near 90% risk of recurrence in subsequent pregnancies.

Case We present a case of a newborn patient delivered to a 32-year-old G2P1000 mother who received prolonged antenatal intravenous immunoglobulin (IVIG) treatment during the current pregnancy due to the neonatal death of the first child from GALD-related liver failure. Postnatal testing, including a liver magnetic resonance imaging (MRI) and buccal biopsy of this newborn, showed normal morphology of the liver without any abnormal iron deposition. Additional laboratory testing showed a lack of any liver injury.

Conclusion This case supports the use of antenatal IVIG immunotherapy to prevent the recurrence of GALD in subsequent pregnancies.

Key Points

• GALD can lead to severe fetal liver injury.

• GALD is highly recurrent in subsequent pregnancies.

• Prophylactic IVIG may prevent GALD recurrence.

Keywords

gestational alloimmune liver disease - neonatal hemochromatosis - antenatal IVIG - recurrence - fetal liver injury

Publication History

Received: 04 June 2024

Accepted: 10 July 2024

Accepted Manuscript online:
05 December 2024

Article published online:
07 January 2025

• References

• 1 Whitington PF. Gestational alloimmune liver disease and neonatal hemochromatosis. Semin Liver Dis 2012; 32 (04) 325-332
• 2 Whitington PF, Kelly S. Outcome of pregnancies at risk for neonatal hemochromatosis is improved by treatment with high-dose intravenous immunoglobulin. Pediatrics 2008; 121 (06) e1615-e1621
• 3 Feldman AG, Whitington PF. Neonatal hemochromatosis. J Clin Exp Hepatol 2013; 3 (04) 313-320
• 4 Whitington PF, Kelly S, Taylor SA. et al. Antenatal treatment with intravenous immunoglobulin to prevent gestational alloimmune liver disease: comparative effectiveness of 14-week versus 18-week initiation. Fetal Diagn Ther 2018; 43 (03) 218-225
• 5 Rand EB, Karpen SJ, Kelly S. et al. Treatment of neonatal hemochromatosis with exchange transfusion and intravenous immunoglobulin. J Pediatr 2009; 155 (04) 566-571
• 6 Whitington PF, Hibbard JU. High-dose immunoglobulin during pregnancy for recurrent neonatal haemochromatosis. Lancet 2004; 364 (9446) 1690-1698
• 7 Larson-Nath C, Vitola BE. Neonatal acute liver failure. Clin Perinatol 2020; 47 (01) 25-39
• 8 Roos Mariano da Rocha C, Rostirola Guedes R, Kieling CO, Rossato Adami M, Cerski CT, Gonçalves Vieira SM. Neonatal liver failure and congenital cirrhosis due to gestational alloimmune liver disease: a case report and literature review. Case Rep Pediatr 2017; 2017: 7432859
• 9 Taylor SA, Kelly S, Alonso EM, Whitington PF. The effects of gestational alloimmune liver disease on fetal and infant morbidity and mortality. J Pediatr 2018; 196: 123-128.e1
• 10 Smith SR, Shneider BL, Magid M, Martin G, Rothschild M. Minor salivary gland biopsy in neonatal hemochromatosis. Arch Otolaryngol Head Neck Surg 2004; 130 (06) 760-763
• 11 Yeh PJ, Huang SF, Chiang MC, Wang CJ, Lai MW. Efficacy of intravenous immunoglobulin/exchange transfusion therapy on gestational alloimmune liver disease. Front Pediatr 2021; 9: 680730
• 12 Flynn DM, Mohan N, McKiernan P. et al. Progress in treatment and outcome for children with neonatal haemochromatosis. Arch Dis Child Fetal Neonatal Ed 2003; 88 (02) F124-F127