Maeda-Tanaka Maki, Haruta Shoji, Sado Toshiyuki, Uchida Yumiko, Takahashi Yukihiro, Asada Hideo, Miyagawa Sachiko
Department of Dermatology, Perinatal Medical Center, Nara Medical University Hospital, Kashihara City, Nara 634-8522, Japan.
J Dermatol. 2006 Oct;33(10):714-8. doi: 10.1111/j.1346-8138.2006.00166.x.
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.
华氏巨球蛋白血症性紫癜（HGP）是一种罕见的慢性疾病，其特征为腿部反复出现紫癜、血清γ球蛋白多克隆增加、红细胞沉降率升高以及类风湿因子阳性。一名患有HGP 14年的30岁初产妇在妊娠25周时被发现胎儿心动过缓。实验室检查显示母体血清中抗Ro/SSA和抗La/SSB抗体呈阳性。39周时进行了剖宫产，一名体重2750克的女婴出生时患有完全性房室传导阻滞。幸运的是，新生儿期平安无事，无需进行起搏治疗。产后母体HGP病情加重，但未经治疗在1周内缓解。医生应意识到HGP患者可能存在与新生儿狼疮相关的抗Ro/SSA和抗La/SSB自身抗体。筛查这些自身抗体很重要，可作为识别和管理高危妊娠的标志物。
