双胎贫血红细胞增多序列症一例及文献复习  

Report of a case with twin anemia-polycythemia sequence and literature review

作　　者：汪吉梅[1] 程国强[2] 钱蓓倩[1] 孟永勤[1] 

机构地区：[1]上海市复旦大学附属妇产科医院新生儿科,200011 [2]复旦大学附属儿科医院新生儿科

出　　处：《中华儿科杂志》2013年第1期21-28,共8页Chinese Journal of Pediatrics

摘　　要：目的探讨双胎贫血红细胞增多序列症的临床表现、产前特点、诊断标准及分级、治疗及近期预后等，以提高临床医生对本病的认识。方法报道1例双胎贫血红细胞增多序列症患儿的临床资料，并对近5年来已报道的15例进行文献复习。结果双胎贫血红细胞增多序列症的报道有增多趋势。产前表现：有13／16例是自然发生的，有3／16例是发生在激光治疗术后的。16例均无羊水量差异。超声多普勒大脑中动脉峰值流速（MCA—PSV）在供血胎大于1．5MoM的有11／16例，3／16例未测；在受血胎小于1．0MoM的有10／16例，3／16例未测。有胎儿水肿的有6／16例。宫内干预：未治疗的9／16例，宫内输血的4／16例，胎儿镜下激光凝固胎盘吻合血管的4／16例，脐带阻塞选择性灭胎的2／16例，1／16例受血儿给予宫内血液稀释治疗。生后表现：贫血儿平均血红蛋白浓度为95g／L，多血质儿为208g／L，胎胎间血红蛋白差异大于80g／L的有10／16例，2／16例小于80g/L（宫内激光治疗后）。5／16例胎胎间网织红细胞比率大于1．7，1／16例小于1．7（宫内激光治疗后），8／16例未提供网织红细胞比率（使用的是网织红细胞绝对计数）。10／16例胎盘彩色染料灌注有直径小于1mm的动静脉吻合支存在，2／16例胎盘无残存的血管吻合支（宫内激光治疗后），2／16例未提供胎盘灌注结果。生后治疗：9／16例供血儿有贫血表现，其中有6／16例予输血治疗；6／16例受血儿有高黏滞血症表现，给予部分换血治疗。11／16例新生儿期神经发育正常，1／16例双胎颅脑超声持续脑室周围白质高回声，本例随访至纠正胎龄3个月时神经发育正常，继续随访中。结论双胎贫血红细胞增多序列症是胎胎输血综合征的一个特殊类型：单绒毛膜双羊膜囊双胎，产前无明显羊水量差异，出生体重相差不大，但血红蛋�Objective To summarize and review the clinical characteristics including clinical features, prenatal characteristics, diagnosis, treatments and short-term outcomes of the twin anemia- polycythemia sequence （TAPS） to improve the recognition of the disease. Method The clinical data of one case with twin anemia-polycythemia sequence and the reports of 15 cases seen in the past 5 years were reviewed and analyzed. Result There was an increasing number of reports of cases with TAPS. Prenatal manifestation： among the 16 cases, TAPS occurred in 13 cases naturally and in 3 cases occurred after laser treatment. Amniotic fluid volume showed no significant difference in 16 cases. Middle cerebral artery peak systolic velocity （MCA-PSV） 〉 1.5 muhiples of the median （MoM） in the donor were 11/16 cases and 3/ 16 cases were not tested. MCA-PSV 〈 1.0 MoM in the recipient were seen in 10/16 cases and in 3/16 cases MCA-PSV was not tested. Hydrops fetalis was found in 6/16 cases. Intrauterine intervention： intrauterine blood transfusion was performed in 4/16 cases, fetoscopic laser occlusion of chorioangiopagus vessels was performed in 4/16 cases, umbilical cord occlusion selective feticide was done in 2/16 cases and intrauterine hemodilution in the recipient was performed in 1/16 case. Postnatal manifestation： average hemoglobin concentration in the anemic neonate was 95 g/L and in the polycythemic one was 208 g/L, intertwin Hb difference was 〉 80 g/L in 10/16 cases and 〈 80 g/L in 2/16 cases （after intrauterine laser treatment）. Intertwin reticulocyte count ratio was 〉 1.7 in 5/16 cases and 〈 1.7 in 1/16 case （ after intrauterine laser treatment）. Postnatal treatment： 9/16 cases of donor had anemia, among them, 6/16 cases were given blood transfusions, 6/16 cases of recipient with hyperviscosity underwent partial exchange transfusions. Neurodevelopmental follow-up during neonatal period was normal in 11/16 cases, in our case, neurodevelopmental follow-up at the corrected gestational age 3 mon

关 键 词：红细胞增多症 双胎输血综合征 双胎疾病 大脑中动脉 双胎贫血红细胞 增多序列症 

分 类 号：R725.5[医药卫生—儿科]