对常规 RHD 和意外抗 D 的 D 阳性镰状细胞病患者的 RHD+ 红细胞进行基因分型。

IF 21 1区 医学 Q1 HEMATOLOGY Blood Pub Date : 2024-11-07 DOI:10.1182/blood.2024025602
Stella T Chou, Julia Mewha, David F Friedman, Victoria Lazariu, Shaimaa Makrm, Gorka Ochoa, Sunitha Vege, Connie M Westhoff
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引用次数: 0

摘要

抗-D 可发生在遗传了编码部分 D 抗原表达的 RHD 基因变体的 D 阳性患者身上,但在具有传统 RHD 基因的镰状细胞病患者的血浆中也会发现意想不到的抗-D,这些患者输注的是来自黑人捐献者的血液。这些抗-D 很可能是由供体细胞上表达的变异型 Rh 刺激的,但抗-D 患者,无论病因如何,终生都要输注 D 阴性(Rh 阴性)血液。因此,Rh 阴性单位的使用量大大增加,尤其是需要长期输血的患者,这可能导致 Rh 阴性血液库存紧张。我们测试了抗 D 阳性且通过 RHD 基因分型检测为常规 RhD 的 D 阳性患者是否可以安全地恢复 D 阳性输血,而不会再次出现抗 D 阳性或使用来自黑人献血者的 RHD 基因型匹配单位影响红细胞存活率。五名接受慢性红细胞置换的患者每次接受的 D 阳性单位数量不断增加,总共输注了 72 个经 RHD 基因分型的 D 阳性单位,没有出现抗 D 再刺激现象。在研究过程中发现,意外的抗 C 和抗 E 与具有变异 RHCE 等位基因的供体有关。对用于输血的 D 阳性献血单位进行 RH 基因分型可改善宝贵的黑人献血单位的使用和分配,减少对 Rh 阴性血液的需求。
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Genotyped RhD+ red cells for D-positive patients with sickle cell disease with conventional RHD and unexpected anti-D.

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.

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来源期刊
Blood
Blood 医学-血液学
CiteScore
23.60
自引率
3.90%
发文量
955
审稿时长
1 months
期刊介绍: Blood, the official journal of the American Society of Hematology, published online and in print, provides an international forum for the publication of original articles describing basic laboratory, translational, and clinical investigations in hematology. Primary research articles will be published under the following scientific categories: Clinical Trials and Observations; Gene Therapy; Hematopoiesis and Stem Cells; Immunobiology and Immunotherapy scope; Myeloid Neoplasia; Lymphoid Neoplasia; Phagocytes, Granulocytes and Myelopoiesis; Platelets and Thrombopoiesis; Red Cells, Iron and Erythropoiesis; Thrombosis and Hemostasis; Transfusion Medicine; Transplantation; and Vascular Biology. Papers can be listed under more than one category as appropriate.
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