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 gamma-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.
Maeda-Tanaka
Maki
M
Department of Dermatology, Perinatal Medical Center, Nara Medical University Hospital, Kashihara City, Nara 634-8522, Japan.
Haruta
Shoji
S
Sado
Toshiyuki
T
Uchida
Yumiko
Y
Takahashi
Yukihiro
Y
Asada
Hideo
H
Miyagawa
Sachiko
S
D000328:Adult; D005260:Female; D006327:Heart Block / Q000151:congenital* / Q000196:embryology; D006801:Humans; D007231:Infant, Newborn; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic; D011694:Purpura, Hyperglobulinemic / Q000150:complications*