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Eculizumab for atypical hemolytic uremic syndrome in pregnancy
发布于：2013年7月29日 文字：【大】【中】【小】
Obstet Gynecol. 2013 Aug;122(2 Pt2):487-9. doi: 10.1097/AOG.0b013e31828e2612.
Eculizumab for atypical hemolytic uremic syndrome in pregnancy.
Ardissino G, Wally Ossola M, Maria Baffero G, Rigotti A, Cugno M.
BACGROUND: Atypical hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy often caused by mutations in complement genes. During pregnancy, disease outcome is poor both for mother and fetus. Since 2009, the humanized monoclonal antibody eculizumab has been successfully used in the treatment of atypical HUS in nonpregnant patients.
CASE: A 26-year-old woman with a homozygous mutation in complement factor H developed a relapse of atypical HUS at 17 weeks of gestation in her first pregnancy. Because the disease remained active despite multiple plasma exchanges, eculizumab was started at 26 weeks of gestation. It was well tolerated and has led to remission and to the delivery of a healthy neonate.
CONCLUSION: Eculizumab may be useful for the treatment of atypical HUS during pregnancy.
妊娠是aHUS的诱因之一；部分aHUS患者可能在妊娠时或分娩后首次发病。2011年以来抗C5单抗（Eculizumab）在多个国家先后被批准用于治疗aHUS。Eculizumab为IgG2/4嵌合体，其通过胎盘屏障的能力与IgG2类似，IgG2是所有 IgG亚型通过胎盘屏障最少的，因此eculizumab通过胎盘很少，而且aHUS活动多发生于妊娠晚期或分娩后，另外在阵发性睡眠性血红蛋白尿（PNH）中的经验是eculizumab在孕妇相对是安全的（C级），因此理论上来说对于妊娠的aHUS患者在向患者及家属充分交代后是可以使用eculizumab的；但之前无相关临床实际应用报道。本文报道了一例在怀孕前即诊断aHUS（之后发现为H因子纯合缺失），在第一次妊娠17周后aHUS复发，经过强化血浆置换病情一度好转，在妊娠26周时开始使用eculizumab（开始每周900mg×2周，之后每2周1200mg至分娩），患者达到缓解并在孕38周后成功分娩一健康婴儿，患者也无明显不良反应。