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镰状细胞病中迟发性溶血性输血反应和高溶血的临床表现。

Clinical presentation of delayed hemolytic transfusion reactions and hyperhemolysis in sickle cell disease.

作者信息

Fasano R M, Miller M J, Chonat S, Stowell S R

机构信息

Center for Transfusion and Cellular Therapies, Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, USA.

Center for Transfusion and Cellular Therapies, Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, USA.

出版信息

Transfus Clin Biol. 2019 May;26(2):94-98. doi: 10.1016/j.tracli.2019.02.002. Epub 2019 Feb 22.

DOI:10.1016/j.tracli.2019.02.002
PMID:30837199
Abstract

Red blood cell (RBC) transfusion therapy is a key component in the comprehensive management of patients with sickle cell disease (SCD). Consequently, most adult SCD patients will receive at least one, and many will receive more than a hundred RBC transfusions in their lifetime. SCD patients develop RBC alloantibodies much more frequently than non-SCD transfused patients, which often make the selection of compatible RBCs extremely difficult, in addition to placing patients at significantly higher risk of suffering from delayed hemolytic transfusion reactions (DHTRs). Similar to alloimunization, DHTRs are much more common in patients with SCD compared to other heavily transfused populations, and are particularly consequential due to their propensity to cause hyperhemolysis, a life-threatening phenomenon in which both transfused RBCs in addition to the patient's own sickle-erythrocytes are destroyed. In this review, we highlight the incidence and pathophysiology of DHTRs; illustrate common presentations, appropriate evaluations and outcomes of DHTRs in patients with SCD; and discuss strategies for preventing or reducing the likelihood of DHTRs from occurring.

摘要

红细胞(RBC)输血治疗是镰状细胞病(SCD)患者综合管理的关键组成部分。因此,大多数成年SCD患者一生中至少会接受一次红细胞输血,许多患者会接受超过一百次的红细胞输血。与非SCD输血患者相比,SCD患者产生红细胞同种抗体的频率要高得多,这不仅常常使选择相容的红细胞变得极其困难,还会使患者遭受迟发性溶血性输血反应(DHTR)的风险显著增加。与同种免疫相似,与其他大量输血人群相比,DHTR在SCD患者中更为常见,并且由于其倾向于导致高溶血(一种危及生命的现象,即输入的红细胞以及患者自身的镰状红细胞都会被破坏)而尤为严重。在本综述中,我们强调了DHTR的发生率和病理生理学;阐述了SCD患者中DHTR的常见表现、适当评估和结果;并讨论了预防或降低DHTR发生可能性的策略。

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