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伊朗镰状细胞病和镰状细胞-地中海贫血病患者的 Rh 血型系统及其在同种免疫率中的作用。

The Rh blood group system and its role in alloimmunization rate among sickle cell disease and sickle thalassemia patients in Iran.

机构信息

Blood Transfusion Research Center, High Institute for Research and Education in Transfusion Medicine, Tehran, Iran.

Thalassemia & Hemoglobinopathy Research Center, Research Institute of Health, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran.

出版信息

Mol Genet Genomic Med. 2021 Mar;9(3):e1614. doi: 10.1002/mgg3.1614. Epub 2021 Feb 6.

DOI:10.1002/mgg3.1614
PMID:33547762
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8104156/
Abstract

INTRODUCTION

The alloimmunization following blood transfusion can be life-threatening. The Rh alloantibodies are one of the most common causes contributing to alloimmunization. This study aimed to evaluate the rate and causes of alloimmunization and to determine the Rh phenotypes and genotypes among sickle cell disease (SCD) and sickle thalassemia (Sβ).

MATERIALS AND METHODS

Our study included 104 SCD and Sβ patients referring to Baghaei 2 Hospital of Ahvaz in 2019 using a non-random simple sampling method. The blood samples were collected for Rh phenotypes, alloantibody screening and identification, and molecular tests. The SSP-PCR and RFLP methods with the Pst 1 enzyme were used.

RESULTS

The alloimmunization rate was 9.6% and 13.2% based on immunohematological tests and medical records, respectively. The main alloantibodies (90%) were anti-Rh, and 40% of the patients had multiple alloantibodies. A significant correlation was found between gender and alloimmunization. The phenotypes of DCce (37.5%), DCcEe (24%), Dce (20.2%), and dce (5.8%) and genotypes of R1r (25%), R1R2 (20.2%), R1R1 (18.3%), and R1R0 (10.6%) were the most prevalent. The R1R2 was a frequent genotype in Sβ.

CONCLUSION

R0r' and R1R0 genotypes were limited to our population in Iran. Due to the differences in RH genotypes between our population and others, the blood transfusion from other ethnicities increased our total alloimmunization rate.

摘要

简介

输血后的同种免疫可能危及生命。Rh 同种抗体是导致同种免疫的最常见原因之一。本研究旨在评估同种免疫的发生率和原因,并确定镰状细胞病 (SCD) 和镰状β地中海贫血 (Sβ) 患者的 Rh 表型和基因型。

材料和方法

我们的研究包括 2019 年使用非随机简单抽样法向阿瓦兹 Baghaei 2 医院就诊的 104 名 SCD 和 Sβ 患者。采集血液样本进行 Rh 表型、同种抗体筛查和鉴定以及分子检测。使用 SSP-PCR 和 Pst 1 酶的 RFLP 方法。

结果

根据免疫血液学检测和病历,同种免疫发生率分别为 9.6%和 13.2%。主要同种抗体(90%)为抗 Rh,40%的患者有多种同种抗体。性别与同种免疫之间存在显著相关性。DCce(37.5%)、DCcEe(24%)、Dce(20.2%)和 dce(5.8%)表型和 R1r(25%)、R1R2(20.2%)、R1R1(18.3%)和 R1R0(10.6%)基因型最为常见。R1R2 是 Sβ 中的常见基因型。

结论

R0r'和 R1R0 基因型在伊朗的我们的人群中是有限的。由于我们的人群与其他人的 RH 基因型存在差异,来自其他种族的输血增加了我们的总同种免疫率。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4dcb/8104156/32733fcec58e/MGG3-9-e1614-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4dcb/8104156/b09cdb3b0e5c/MGG3-9-e1614-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4dcb/8104156/32733fcec58e/MGG3-9-e1614-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4dcb/8104156/b09cdb3b0e5c/MGG3-9-e1614-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4dcb/8104156/32733fcec58e/MGG3-9-e1614-g001.jpg

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