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地中海贫血和骨髓增生异常综合征患者的真实血液管理实践

Real Life Blood Management Practices in Thalassemia and Myelodysplastic Syndrome Patients.

作者信息

Aydin Muruvvet S, Cengiz Esra

机构信息

Department of Hematology and Bone Marrow Transplantation, Ankara Sehir Hastanesi, Ankara, Turkey.

Department of Hematology, Mehmet Akif Inan Egitim Ve Arastirma Hastanesi, Sanliurfa, Turkey.

出版信息

Anemia. 2025 Jan 8;2025:7257391. doi: 10.1155/anem/7257391. eCollection 2025.

Abstract

The effect of pretransfusion hemoglobin on transfusion burden, thrombosis, and mortality in thalassemia and myelodysplastic syndrome is unclear. We aimed to study the pretransfusion hemoglobin and erythrocyte transfusion burden and investigate the effects of these variables on each other in real-life in thalassemia and myelodysplastic syndrome. Adult patients with thalassemia and myelodysplastic syndrome who received at least one erythrocyte concentrate unit outpatient at Sanliurfa Mehmet Akif Inan Training and Research Hospital during 1 year were included in the study. The data were retrospectively obtained. Ethical approval was obtained for the study. Ninety-two patients were included in the study. In thalassemia major, pretransfusion hemoglobin ≥ 9 g/dL was associated with a lower median annual number of transfused erythrocyte concentrate units (15 vs. 27) and median annual number of transfusion sessions (11 vs. 14) =0.002, =0.009, respectively). In myelodysplastic syndrome, a pretransfusion hemoglobin level ≥ 8 g/dL was associated with a lower median annual number of transfused erythrocyte concentrate units (6 vs. 24) (=0.016). In thalassemia major with an intact spleen, pretransfusion hemoglobin ≥ 8 g/dL was associated with an increased median annual number of transfused erythrocyte concentrate units (32 vs. 27) and median annual number of transfusion sessions (18 vs. 14) (=0.046, =0.038, respectively). In conclusion, higher pretransfusion hemoglobin levels were related to a lower transfusion burden in thalassemia major and myelodysplastic syndrome.

摘要

输血前血红蛋白对地中海贫血和骨髓增生异常综合征患者的输血负担、血栓形成及死亡率的影响尚不清楚。我们旨在研究输血前血红蛋白水平与红细胞输血负担,并在现实生活中调查这些变量在地中海贫血和骨髓增生异常综合征患者中相互之间的影响。纳入了在1年期间于尚勒乌尔法穆罕默德·阿基夫·伊南培训与研究医院门诊接受至少1个单位红细胞浓缩液输注的成年地中海贫血和骨髓增生异常综合征患者。数据通过回顾性方式获取。本研究获得了伦理批准。92例患者纳入本研究。在重型地中海贫血中,输血前血红蛋白≥9 g/dL与较低的红细胞浓缩液单位年输注中位数(15比27)和较低的年输血次数中位数(11比14)相关(分别为P = 0.002,P = 0.009)。在骨髓增生异常综合征中,输血前血红蛋白水平≥8 g/dL与较低的红细胞浓缩液单位年输注中位数(6比24)相关(P = 0.016)。在脾脏完整的重型地中海贫血患者中,输血前血红蛋白≥8 g/dL与较高的红细胞浓缩液单位年输注中位数(32比27)和较高的年输血次数中位数(18比14)相关(分别为P = 0.046,P = 0.038)。总之,较高的输血前血红蛋白水平与重型地中海贫血和骨髓增生异常综合征患者较低的输血负担相关。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bdd1/11735053/8cf13f501758/ANEMIA2025-7257391.001.jpg

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