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为常规 RHD 和意外抗-D 的镰状细胞病 D 阳性患者进行 RhD+ 基因型红细胞配型。

Genotyped RhD+ red cells for D-positive patients with sickle cell disease with conventional RHD and unexpected anti-D.

机构信息

Division of Hematology, Department of Pediatrics, University of Pennsylvania, The Children's Hospital of Philadelphia, Philadelphia, PA.

Division of Transfusion Medicine, Department of Pathology and Laboratory Medicine, University of Pennsylvania, The Children's Hospital of Philadelphia, Philadelphia, PA.

出版信息

Blood. 2024 Nov 7;144(19):2045-2049. doi: 10.1182/blood.2024025602.

DOI:10.1182/blood.2024025602
PMID:39172743
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11561532/
Abstract

Anti-D can occur in D-positive patients who inherit RHD genetic variants encoding partial D antigen expression, but unexpected anti-D is also found in the plasma of patients with sickle cell disease who have conventional RHD gene(s) and are transfused with units from Black donors. These anti-D are likely stimulated by variant Rh expressed on donor cells; however, patients with anti-D, regardless of cause, are transfused for a lifetime with D-negative (Rh-negative) blood. This results in significant increased use of Rh-negative units, especially for those requiring chronic transfusion, which can strain Rh-negative blood inventories. We tested whether D-positive patients who made anti-D and had conventional RhD by RHD genotyping could safely be returned to D-positive transfusions without anti-D reappearance or compromised red blood cell survival using RHD genotype-matched units from Black donors. Five patients receiving chronic red cell exchange received an increasing number of D-positive units per procedure with a total of 72 D-positive RHD genotyped units transfused, with no anti-D restimulation. Unexpected anti-C and anti-E were identified during the study associated with donors with variant RHCE alleles. RH genotyping of D-positive units for transfusion may improve use and allocation of valuable Black donor units and reduce demand for Rh-negative blood. This trial was registered at www.clinicaltrials.gov as NCT04156906.

摘要

抗-D 可发生于遗传了部分 D 抗原表达的 RHD 基因变异体的 D 阳性患者中,但在具有常规 RHD 基因且输注来自黑人供者血液的镰状细胞病患者的血浆中也会意外发现抗-D。这些抗-D 可能是由供体细胞上表达的变异 Rh 刺激产生的;然而,无论原因如何,有抗-D 的患者都需要终生输注 D 阴性(Rh 阴性)血液。这导致对 Rh 阴性单位的大量需求增加,尤其是对那些需要长期输血的患者,这可能会使 Rh 阴性血液库存紧张。我们通过对 D 阳性患者进行 RHD 基因分型,检测了那些产生抗-D 且具有常规 RhD 的患者是否可以安全地重新输注 D 阳性血液而不出现抗-D 再次出现或红细胞存活率降低的情况,方法是使用来自黑人供者的 RHD 基因型匹配单位。5 名接受慢性红细胞置换的患者在每次手术中接受的 D 阳性单位数量逐渐增加,共输注了 72 个经 D 阳性 RHD 基因分型的单位,没有出现抗-D 再次刺激。在研究过程中,发现了意外的抗-C 和抗-E,与具有变异 RHCE 等位基因的供者有关。对用于输血的 D 阳性单位进行 RH 基因分型可能会改善宝贵的黑人供者单位的使用和分配,并减少对 Rh 阴性血液的需求。该试验在 www.clinicaltrials.gov 上注册,编号为 NCT04156906。

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本文引用的文献

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Transfusing children with sickle cell disease using blood group genotyping when the pool of Black donors is limited.在黑人供者血源有限时,对镰状细胞病患儿进行血型基因定型输血。
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具有变异RH基因型的献血者与意外的Rh抗体相关的证据。
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