Transient Neonatal Liver Disease After Maternal Antenatal Intravenous Immunoglobulins in Gestational Alloimmune Liver Disease Associated With Neonatal Hemochromatosis

OBJECTIVES:: Neonatal hemochromatosis is a rare gestational disease resulting in severe fetal liver disease with extrahepatic iron overload sparing the reticuloendothelial system. Recurrrence can be prevented with intravenous immunoglobulin (IVIG) infusions during pregnancy supporting an alloimmune etiology. Our study aimed to assess the effect of antenatal treatment with IVIG on the outcome of pregnancies in women with a past history of documented neonatal hemochromatosis likely due to gestational alloimmune disease (GALD), and to analyse IVIG tolerance. METHODS:: From 2004 to 2012, 8 pregnant women were treated with IVIG at 1 g/kg body weight weekly from 18 weeks' gestation until birth in a prospective multicentre study. RESULTS:: All 8 neonates born from treated women survived. Five developed mild neonatal liver disease with hepatomegaly (n = 1), hyperechogenic liver (n = 2), abnormal liver function tests (n = 1), raised serum ferritin (n = 3) and alphafetoprotein (n = 5) levels, or mild iron overload on liver magnetic resonance imaging (MRI) (n = 1). Ferritin and alphafetoprotein levels normalised before 14 days and 2 months respectively. A per-mother basis analysis comparing outcomes of treated (n = 8) and untreated (n = 9) gestations showed a significant improvement in survival of neonates with gestational IVIG therapy (survival 8/8 vs 0/9; P \textless 0.001). Side effects of IVIG occurred in 5 mothers leading to discontinuation of treatment in 1 case. Preterm neonates born before 37 weeks' gestation had a decreased risk of neonatal liver disease (P = 0.04). CONCLUSIONS:: Antenatal treatment with IVIG in women at risk for GALD recurrence improves the outcome of pregnancies despite mild signs of transient neonatal liver disease.