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土耳其血红蛋白病患者红细胞输血治疗与免疫血液学服务调查

Survey of Red Cell Transfusion Therapy and Immunohematology Services for Patients with Hemoglobinopathies in Türkiye.

作者信息

Gürlek Gökçebay Dilek, Andıç Neslihan, Flegel Willy Albert

机构信息

NIH Clinical Center, National Institutes of Health, Department of Transfusion Medicine, Bethesda, USA

Ankara Bilkent City Hospital, Department of Pediatric Hematology and Oncology, Ankara, Türkiye

出版信息

Turk J Haematol. 2025 Aug 29;42(3):196-202. doi: 10.4274/tjh.galenos.2025.2025.0133. Epub 2025 Jul 23.

Abstract

OBJECTIVE

Transfusion therapy is critical for many patients with β-thalassemia or sickle cell disease (SCD). We aimed to review current practices and document chronic transfusion therapy for patients with hemoglobinopathies in the transfusion service centers of Türkiye.

MATERIALS AND METHODS

A survey with 16 structured questions was distributed electronically to adult and pediatric hematologists in Türkiye. Responses were received from 37 centers across 18 cities, representing 1449 patients diagnosed with β-thalassemia major, β-thalassemia intermedia, and SCD.

RESULTS

Although 79% of centers reported performing extended red cell antigen typing prior to the first transfusion, adherence to national transfusion guidelines was inconsistent. Only 16% of centers routinely performed indirect antiglobulin testing before each transfusion despite guideline recommendations. Antibody identification capabilities varied, with 26% of centers lacking the capability onsite. Elution and adsorption testing were always performed at 13% of centers only, predominantly including university hospitals. Nearly half of the centers were always able to provide D, C, E, c, e, and Kell compatible red cell units, but one-fourth reported that they were unable to consistently provide compatible units due to limited supply. There was no access to red cell genotyping in the country.

CONCLUSION

Our survey revealed disparities in transfusion practices and transfusion service laboratory infrastructure across Türkiye. There is a need for national policy initiatives to mandate adherence to national and international guidelines, expand immunohematology testing capabilities, and ensure the equitable distribution of phenotype-matched red cell units. These findings will contribute to discussions on establishing a centralized immunohematology reference laboratory and enabling red cell genotyping within the country to improve transfusion safety and health equity in hemoglobinopathy care.

摘要

目的

输血治疗对许多β地中海贫血或镰状细胞病(SCD)患者至关重要。我们旨在回顾土耳其输血服务中心对血红蛋白病患者进行慢性输血治疗的当前做法并记录相关情况。

材料与方法

向土耳其的成人和儿科血液科医生电子分发了一份包含16个结构化问题的调查问卷。收到了来自18个城市37个中心的回复,这些回复代表了1449名被诊断为重型β地中海贫血、中间型β地中海贫血和SCD的患者。

结果

尽管79%的中心报告在首次输血前进行了扩展红细胞抗原分型,但对国家输血指南的遵守情况并不一致。尽管有指南建议,但只有16%的中心在每次输血前常规进行间接抗球蛋白试验。抗体鉴定能力各不相同,26%的中心现场缺乏该能力。洗脱和吸附试验仅在13%的中心进行,主要是大学医院。近一半的中心始终能够提供D、C、E、c、e和Kell血型相合的红细胞单位,但四分之一的中心报告称,由于供应有限,他们无法始终提供相合的单位。该国无法进行红细胞基因分型。

结论

我们的调查揭示了土耳其各地输血实践和输血服务实验室基础设施存在差异。需要采取国家政策举措,强制遵守国家和国际指南,扩大免疫血液学检测能力,并确保表型匹配的红细胞单位公平分配。这些发现将有助于讨论建立一个集中的免疫血液学参考实验室以及在国内开展红细胞基因分型,以提高血红蛋白病治疗中的输血安全性和健康公平性。

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