Fatal hydrops fetalis caused by anti-D in a mother with partial D

BACKGROUND: Rare cases of hemolytic disease of the newborn occur in women with genetic variants of the D antigen. A partial D variant might be suspected when typing for the D antigen shows weaker-than-normal reactions (weak D). Historically, patients with a weak D phenotype have not been considered candidates for Rh immune globulin prophylaxis. CASE: A gravida 2, para 1 woman with A-positive blood type, delivered an infant who died from severe hemolytic disease of the newborn 6 days after birth. Anti-D, undetectable at the first prenatal visit, was identified (titer 1:64) at delivery. The mother's red cells were partial D(VI) phenotype. CONCLUSION: Although severe hemolytic disease in patients with partial D is rare, this neonatal death illustrates the need for a change in management of women with weak D.