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Ethical Issues and Management of Fetal Hemolytic Anemia Caused by Anti-Rh17 in a Multipara with Rare –D– Phenotype
Transfusion Medicine and Hemotherapy ( IF 2.2 ) Pub Date : 2021-01-05 , DOI: 10.1159/000513124
Patrick P Torreiter 1 , Susanne Macher 1 , Eva-Maria Matzhold 1 , Bernhard Resch 2 , Philipp Klaritsch 3 , Günther F Körmöczi 4 , Helene Polin 5 , Leopold Neuhold 6 , Marlies Schönbacher 4 , Peter Schlenke 1 , Thomas Wagner 1
Affiliation  

Background: The development of allo-anti-Rh17 (anti-Hr0) in a –D– phenotype whose red blood cells (RBCs) lack CcEe antigens is most likely triggered by transfusion, transplantation, or pregnancy. Gene conversion is the predominating factor in generating RHD-CE-D and RHCE-D-CE hybrids like –D–. Methods: We report here immunohematological and obstetrical data from 2 of the 5 pregnancies of a 24-year-old woman presenting with the –D– phenotype with anti-Rh17. Blood group typing, antibody screening, antibody differentiation, direct antiglobulin test (DAT), and antibody titers were performed by routine gel technology and tube testing. Additionally, molecular genetic analysis was performed. Fetal surveillance was done by sonographic evaluation of the fetal middle cerebral artery peak systolic velocity (MCA-PSV). Results: Blood group typing showed O, C-c-D+E-e- and the DAT was negative. DNA sequencing revealed homozygosity for an RHCE-D(3–9)-CE null allele. Anti-Rh17 titers in the fourth pregnancy remained between 1:8 and 1:128, and no signs for a fetal anemia were observed. However, in the fifth pregnancy, the antibody titers increased up to 1:4,096. Signs of moderate fetal anemia were detected and cesarean section was performed at 34 + 6 weeks of gestation. The newborn presented with hemolytic anemia (cord blood hemoglobin [Hb] = 8.5 mg/dL). She received 2 compatible (small) packed RBC concentrates, phototherapy, and intravenous immunoglobulins. Conclusion: Our case shows that the risk for hemolytic complications increases with the number of pregnancies of sensitized women. Only people who also lack CcEe antigens are compatible as donors. The role of such rare donors as lifesavers, their freedom, and voluntariness conflict with the urgent need for compatible blood.
Transfus Med Hemother


中文翻译:

罕见的-D-表型多对中抗Rh17引起的胎儿溶血性贫血的伦理问题和处理

背景:异体抗Rh17(抗Hr0)在红细胞(RBC)缺乏CcEe抗原的-D-表型中的发展很可能是由输血,移植或妊娠引起的。基因转换是生成–D–等RHD-CE-D和RHCE-D-CE杂种的主要因素。方法:我们在这里报告了来自24岁女性的5例孕妇中的2例的免疫血液学和产科数据,这些孕妇表现出具有抗Rh17的–D–表型。通过常规的凝胶技术和试管测试进行血型分型,抗体筛选,抗体分化,直接抗球蛋白测试(DAT)和抗体滴度。另外,进行了分子遗传分析。通过超声检查胎儿大脑中动脉的收缩期峰值速度(MCA-PSV)进行胎儿监护。结果:血型分型显示O,Cc-D + Ee-,而DAT阴性。DNA测序显示RHCE-D(3-9)-CE的纯合性等位基因无效。在第四次妊娠中,抗Rh17滴度保持在1:8至1:128之间,未观察到胎儿贫血的迹象。但是,在第五次怀孕中,抗体效价增加至1:4,096。检测到中度胎儿贫血的迹象,并在妊娠34 + 6周时进行剖宫产。新生儿出现溶血性贫血(脐血血红蛋白[Hb] = 8.5 mg / dL)。她接受了2种兼容(小)包装的RBC浓缩液,光疗和静脉注射免疫球蛋白。结论:我们的案例表明,溶血并发症的风险随着致敏妇女怀孕次数的增加而增加。只有也缺乏CcEe抗原的人才能作为供体。像救生员这样的稀有捐助者的作用,他们的自由和自愿性与对兼容血液的迫切需求相冲突。
Transfus Med Hemother
更新日期:2021-01-05
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