1Hemocentro Unicamp, Campinas, São Paulo, Brazil.
2Sultan Qaboos University Hospital, Al-khod, Sultanate of Oman.
Immunohematology. 2024 Oct 4;40(3):93-99. doi: 10.2478/immunohematology-2024-0014. Print 2024 Sep 1.
Many Omani patients with sickle cell disease (SCD) undergo red blood cell (RBC) transfusions that are only matched for ABO and D, making RBC alloimmunization a significant concern in this population. Currently, the integration of molecular assays and hemagglutination testing helps to determine RBC phenotypes and genotypes, facilitating the provision of compatible blood and minimizing additional alloimmunization risks in patients with SCD. Based on this finding, our objective was to use molecular methods to predict the extended antigen profile of Omani patients with SCD across various blood group systems including Rh, Kell, Duffy, Kidd, Colton, Lutheran, Dombrock, Diego, Cartwright, and Scianna. This approach aims to implement RBC matching strategies and enhance daily transfusion practices for these patients. Molecular methods encompassed multiplex polymerase chain reaction for BeadChip arrays for variants of and and ID CORE XT for the primary allelic variants of RBCs. This study enrolled 38 patients with SCD, comprising 34 patients with homozygous HbSS, 1 patient with HbSC, and 3 patients with HbS Oman. The predominant ABO blood group was group O, observed in 44.7 percent of patients, followed by group A in 21.1 percent and group B in 13.2 percent. The most prevalent Rh phenotype predicted from the genotype was D+C+E-c+e+, identified in 34.2 percent of patients. All patient samples were K-, exhibiting the k+ Kp(b+) Js(b+) phenotype, with 81.6 percent demonstrating Fy(a-b-) due to the homozygous genotype and 28.9 percent displaying Jk(a+b-). variant alleles were detected in five patients (13.2 %), with only one type of variant () and one type of variant () identified. Alloantibodies were present in 26 patients (68.4%). This study presents the initial comprehensive report of extended RBC antigen profiling in Omani patients with SCD, revealing disparities in the prevalence of RBC phenotypes compared with SCD patients from other regions and countries. Furthermore, our findings underscore a high rate of alloimmunization in these patients, emphasizing the need to implement antigen-matching programs to improve daily transfusion practices.
许多阿曼镰状细胞病（SCD）患者接受仅针对 ABO 和 D 进行匹配的红细胞（RBC）输注，这使得 RBC 同种免疫成为该人群的一个重要关注点。目前，分子分析和血凝检测的整合有助于确定 RBC 表型和基因型，为 SCD 患者提供相容的血液并最大程度地降低额外的同种免疫风险。基于这一发现，我们的目标是使用分子方法来预测阿曼 SCD 患者在各种血型系统（包括 Rh、Kell、Duffy、Kidd、Colton、Lutheran、Dombrock、Diego、Cartwright 和 Scianna）中的扩展抗原谱。这种方法旨在为这些患者实施 RBC 匹配策略并加强日常输血实践。分子方法包括用于 和 变体的多重聚合酶链反应和用于 RBC 主要等位基因变体的 ID CORE XT 的 BeadChip 阵列。这项研究纳入了 38 名 SCD 患者，其中 34 名患者为纯合子 HbSS，1 名患者为 HbSC，3 名患者为 HbS Oman。主要的 ABO 血型群体是 O 型，在 44.7%的患者中观察到，其次是 A 型（21.1%）和 B 型（13.2%）。从基因型预测的最常见 Rh 表型是 D+C+E-c+e+，在 34.2%的患者中观察到。所有患者样本均为 K-，表现出 k+ Kp(b+) Js(b+)表型，由于纯合子 基因型，81.6%的患者显示 Fy(a-b-)，28.9%的患者显示 Jk(a+b-)。在 5 名患者（13.2%）中检测到 变体等位基因，只有一种类型的 变体（）和一种类型的 变体（）。26 名患者（68.4%）存在同种抗体。本研究首次全面报告了阿曼 SCD 患者的 RBC 抗原扩展谱，揭示了与来自其他地区和国家的 SCD 患者相比，RBC 表型的流行率存在差异。此外，我们的研究结果强调了这些患者同种免疫发生率较高，需要实施抗原匹配计划以改善日常输血实践。
