Kirkegaard Julie, Rahorst Lynsi, Burgos Anna, Music Aplenc Lejla, Iqbal Nazia Tabassum, Vege Sunitha, Francis Christine Lomas, Westhoff Connie M
Immunohematology Reference Laboratory, Division of New York Blood Center Enterprises, Community Blood Center, Kansas City, Missouri, USA.
Immunohematology and Genomics Laboratory, New York Blood Center Enterprises, Long Island City, New York, USA.
Transfusion. 2023 Oct;63(10):1962-1968. doi: 10.1111/trf.17525. Epub 2023 Sep 1.
We previously reported unexpected Rh antibodies in the plasma of patients with sickle cell disease (SCD) that demonstrated common Rh specificities in the absence of transfusion of RBCs positive for that antigen. We hypothesize that these antibodies might result from transfusion of antigen-negative donor units with variant RH genotypes.
Plasma testing by tube and IgG gel, extended RBC phenotyping, and HEA and RH genotyping were by standard methods.
A 6-year-old female with SCD, phenotype D + C-c + E-e + K- undergoing exchange transfusion with CEK- and Fy(a-) units, presented with anti-C in the plasma, a + DAT and warm autoantibody (WAA) in the eluate. Her RH genotype was unremarkable: RHDD/DAU0 and RHCEce/ce(48C). Units (n = 10) transfused over the prior 6 months were confirmed CEK- by serology and DNA testing. Most (n = 7) were Rh-negative. A unit with variant RH, RHDDIIIa/weak partial 4.0, RHCEceVS.03/ceVS.02, was transfused 5 weeks prior. Anti-C and + DAT continued to demonstrate for 25 weeks. Total hemoglobin and % Hgb S did not deviate from her established baseline.
We show direct association of plasma anti-C with transfusion of a C-negative unit with variant RH encoding partial D and uncommon V/VS+ hr - phenotype. The antibody was transient, without evidence of compromised survival of transfused RBCs. The +DAT and WAA complicated workups and selection of units, and it is uncertain whether donors of the same genotype should be avoided. Minority donors are important for CEK-matching to avoid depleting Rh-negative supplies. Consideration of patient and donor RH genotypes may avoid unexpected antibodies and improve allocation of rare donations.
我们之前报道过镰状细胞病（SCD）患者血浆中出现意外的Rh抗体，这些抗体在未输注该抗原阳性红细胞的情况下表现出常见的Rh特异性。我们推测这些抗体可能源于输注了具有变异RH基因型的抗原阴性供者单位血液。
采用标准方法进行试管和IgG凝胶血浆检测、扩展红细胞表型分析以及HEA和RH基因分型。
一名6岁患有SCD的女性，表型为D + C-c + E-e + K-，正在接受CEK-和Fy(a-)单位血液的换血治疗，其血浆中出现抗-C，洗脱液中有阳性直接抗球蛋白试验（+DAT）和温自身抗体（WAA）。她的RH基因型无异常：RHDD/DAU0和RHCEce/ce(48C)。在之前6个月内输注的单位血液（n = 10）经血清学和DNA检测确认为CEK-。大多数（n = 7）为Rh阴性。在5周前输注了一个具有变异RH的单位血液，RHDDIIIa/弱部分型4.0，RHCEceVS.03/ceVS.02。抗-C和+DAT持续存在25周。总血红蛋白和血红蛋白S百分比未偏离其既定基线。
我们显示血浆抗-C与输注一个编码部分D和罕见V/VS+ hr -表型的变异RH的C阴性单位血液直接相关。该抗体是短暂的，没有证据表明输注的红细胞存活受到影响。+DAT和WAA使检查和单位血液选择变得复杂，并且不确定是否应避免相同基因型的供者。少数族裔供者对于CEK配型很重要，以避免Rh阴性血液供应枯竭。考虑患者和供者的RH基因型可能避免意外抗体的产生，并改善稀有血液捐赠的分配情况。
