High Dose Immunoglobulin During Pregnancy For Recurrent Neonatal Hemochromatosis      02/01/05

Peter F. Whitington, MD

Department of Pediatrics

The Feinberg School of Medicine of Northwestern University

Children's Memorial Hospital

Chicago, IL

 

Judith U. Hibbard, MD

Department of Obstetrics and Gynecology

The Pritzker School of Medicine

The University of Chicago

Chicago, IL

Abstract

Background: Neonatal hemochromatosis (NH) is a rare disease of gestation that results in severe fetal liver injury. We hypothesized an alloimmune etiology for NH based on its high recurrence rate in sibships. In this study, we assessed the effectiveness of administering high-dose intravenous immunoglobulin derived from pooled serum of multiple donors (IV-Ig) during pregnancy to prevent or alter the severity of recurrent NH.

Study design: Women whose most recent pregnancy ended in documented NH were treated with IV-Ig, 1 g/kg body weight weekly from the 18^th week until the end of gestation. The outcomes of treated pregnancies were compared to the outcomes of randomly selected previous affected pregnancies for each woman, which were used as historical controls.

Results: Fifteen women were treated through 16 pregnancies. All pregnancies progressed uneventfully and resulted in live babies with normal physical examinations and birth weights that were appropriate for gestational age. Twelve babies had evidence of liver involvement with NH: eleven had elevated serum a-fetoprotein and ferritin levels or elevated serum a-fetoprotein, including 4 with coagulopathy (INR > 1.5), and one had coagulopathy alone. All babies survived with medical or no treatment and are currently healthy. When analyzed on a per-mother basis comparing outcomes of treated gestations to randomly selected previous affected gestations, gestational IV-Ig therapy was associated with improved infant survival (p = 0·0009).

Conclusions: Treatment with high-dose IV-Ig during gestation appears to have modified recurrent NH so that it was not lethal to the fetus or newborn. These results further suggest an alloimmune mechanism for recurrent NH.

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