Juvenile‐onset hypergammaglobulinemic purpura and fetal congenital heart block

Waldenström's hypergammaglobulinemic purpura (HGP) is a rare chronic disorder characterized by recurrent purpura on the legs, a polyclonal increase in serum γ‐globulin, an elevated erythrocyte sedimentation rate and a positive rheumatoid factor. A 30‐year‐old primigravid woman with 14 years of HGP was found to have fetal bradycardia at 25 weeks’ gestation. Laboratory investigations demonstrated positive anti‐Ro/SSA and anti‐La/SSB antibodies in the maternal serum. Cesarean delivery was performed at 39 weeks, and a 2750‐g female infant was born with complete atrioventricular block. Fortunately, the neonatal period has been uneventful without need for pace‐making. Maternal HGP exacerbated just after delivery, but resolved within 1 week without treatment. Physicians should be aware of the possible presence of neonatal lupus‐related anti‐Ro/SSA and anti‐La/SSB autoantibodies in patients with HGP. Screening for these autoantibodies is important and could be used as a marker to identify and manage high‐risk pregnancies.